NUTRITIONAL KNOWLEDGE IN PHENYLKETONURIA (PKU)
<p align="justify">ABSTRACT: A review of phenylketonuria (PKU) an autosomal recessive genetic desorder discovered and described in 1934 is presented and discussed. Excess phenylalanine is transamined and its presence or metabolites may cause brain damage. Hydroxylation is the...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Universidade Estadual Paulista
2009-07-01
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| Series: | Alimentos e Nutrição |
| Subjects: | |
| Online Access: | http://serv-bib.fcfar.unesp.br/seer/index.php/alimentos/article/view/730 |
| Summary: | <p align="justify">ABSTRACT: A review of phenylketonuria (PKU) an autosomal recessive genetic desorder discovered and described in 1934 is presented and discussed. Excess phenylalanine is transamined and its presence or metabolites may cause brain damage. Hydroxylation is the most important determinant of phenylalanine homeostasis in humans. It has been shown a great geographic and ethnic variation in the presence of PKU. Neonatal screening is the main method to detect babies with this desorder. Restriction of phenylalanine intake is the most effective PKU treatment. There are several well balanced commercial formulas low in this amino acid. They should be introduced as soon as the positive PKU test is confirmed and be kept for a long time. Present knowledge allows the possibility of pregnancy of PKU girls, under dietary control. Alternative treatments have been proposed for PKU and great advance has lately been achieved on the genetic and nutricional aspects of the disease. KEYWORDS: Phenylketonuria; children; phenylalanine, nutrition PKU. |
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| ISSN: | 0103-4235 2179-4448 |