منابع مشابه
Dietary intervention in the management of phenylketonuria: current perspectives
Phenylketonuria (PKU) is a well-described inborn error of amino acid metabolism that has been treated for >60 years. Enzyme deficiency causes accumulation of phenylalanine (Phe) and if left untreated will lead to profound and irreversible intellectual disability in most children. Traditionally, it has been managed with a low-Phe diet supplemented with a Phe-free protein substitute although newe...
متن کاملThe Use of Glycomacropeptide in Dietary Management of Phenylketonuria
Dietary therapy is the most common therapy applied in treatment of Phenylketonuria (PKU) with restriction of intake of most natural proteins that are rich in Phenylalanine (Phe). Recently, it has been claimed that caseinoglycomacropeptide (GMP), derived of whey, may be used to replace the amino acid formulae (AAF). The Aim of Work. To study the feasibility of use of GMP for partial replacement ...
متن کاملRecommendations on the dietary management of phenylketonuria. Report of Medical Research Council Working Party on Phenylketonuria.
متن کامل
The dietary treatment of phenylketonuria.
Phenylketonuria is an inborn error of the metabolism of phenylalanine associated almost invariably with grave mental deficiency, and often with epilepsy resembling petit mal. It was suggested that the mental deficiency was due to an intoxication by phenylalanine or one of its metabolites and might be relieved by feeding a diet low in phenylalanine (Woolf and Vulliamy, 1951). An economically pra...
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ژورنال
عنوان ژورنال: Proceedings of the Nutrition Society
سال: 1979
ISSN: 0029-6651,1475-2719
DOI: 10.1079/pns19790054