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Titolo:
Phenylketonuria: tyrosine supplementation in phenylalanine-restricted diets
Autore:
van Spronsen, FJ; van Rijn, M; Bekhof, J; Koch, R; Smit, PGA;
Indirizzi:
Univ Groningen Hosp, Beatrix Childrens Hosp, Dept Metab Dis, NL-9700 RB Groningen, Netherlands Univ Groningen Hosp Groningen Netherlands NL-9700 RBningen, Netherlands Childrens Hosp Los Angeles, Div Med Genet, Los Angeles, CA 90027 USA Childrens Hosp Los Angeles Los Angeles CA USA 90027 Angeles, CA 90027 USA
Titolo Testata:
AMERICAN JOURNAL OF CLINICAL NUTRITION
fascicolo: 2, volume: 73, anno: 2001,
pagine: 153 - 157
SICI:
0002-9165(200102)73:2<153:PTSIPD>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
NEUTRAL AMINO-ACIDS; MATERNAL PHENYLKETONURIA; PLASMA PHENYLALANINE; PROTEIN-INTAKE; ORAL TRACER; YOUNG MEN; PREGNANCY; CHILDREN; ADULTS; PKU;
Keywords:
phenylketonuria; PKU; tyrosine; protein substitute; amino acid supplementation; tyrosine supplementation; maternal PKU;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
58
Recensione:
Indirizzi per estratti:
Indirizzo: van Spronsen, FJ Univ Groningen Hosp, Beatrix Childrens Hosp, Dept Metab Dis, POB 30-001, NL-9700 RB Groningen, Netherlands Univ Groningen Hosp POB 30-001 Groningen Netherlands NL-9700 RB
Citazione:
F.J. van Spronsen et al., "Phenylketonuria: tyrosine supplementation in phenylalanine-restricted diets", AM J CLIN N, 73(2), 2001, pp. 153-157

Abstract

Treatment of phenylketonuria (PKU) consists of restriction of natural protein and provision of a protein substitute that lacks phenylalanine but is enriched in tyrosine. Large and unexplained differences exist, however, in the tyrosine enrichment of the protein substitutes. Furthermore, some investigators advise providing extra free tyrosine in addition to the tyrosine-enriched protein substitute, especially in the treatment of maternal PKU. In this article, we discuss tyrosine concentrations in blood during low-phenylalanine, tyrosine-enriched diets and the implications of these blood tyrosine concentrations for supplementation with tyrosine. We conclude that the present method of tyrosine supplementation during the day is far from optimal because it dues not prevent low blood tyrosine concentrations, especiallyafter an overnight fast, and may result in largely increased blood tyrosine concentrations during the rest of the day. Both high tyrosine enrichment of protein substitutes and extra free tyrosine supplementation may not be as safe as considered at present, especially to the fetus of a woman with PKU. The development of dietary compounds that release tyrosine more slowly could be beneficial. We advocate decreasing the tyrosine content of protein substitutes to approximate to6% by wt (6 g/100 g protein equivalent) at most and not giving extra free tyrosine without knowing the diurnal variationsin the blood tyrosine concentration and having biochemical evidence of a tyrosine deficiency. We further advocate that a better daily distribution ofthe protein substitute be achieved by improving the palatability of these products.

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Documento generato il 19/09/20 alle ore 17:29:56