Management of Phenylketonuria and Hyperphenylalaninemia
نویسندگان
چکیده
منابع مشابه
Management of phenylketonuria and hyperphenylalaninemia.
Hyperphenylalaninemia (HPA) is the most frequently inherited disorder of amino acid metabolism (prevalence 1:10,000). In France, a nationwide neonatal screening was organized in 1978 to control its efficacy and patient follow-up. Phenylketonuria (PKU) was diagnosed in 81.6% of screened patients, the remaining affected with either non-PKU HPA (17.2%) or with cofactor deficiency (1.1%). French gu...
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15 صفحه اولTetrahydrobiopterin responsiveness in a series of 53 cases of phenylketonuria and hyperphenylalaninemia in Iran
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Aspartame (N-aspartyl-phenylalanine methyl ester), a widely used artificial sweetener, is hydrolyzed in the intestinal lumen to methanol and to its constituent amino .acids phenylalanine and aspartic acid. Ingestion of this sweetener causes a sharp increase in plasma phenylalanine I vels in normal persons. and in patients with phenylketonu ia.2The label on aspartame-containing products includes...
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ژورنال
عنوان ژورنال: The Journal of Nutrition
سال: 2007
ISSN: 0022-3166,1541-6100
DOI: 10.1093/jn/137.6.1561s