Dietary interventions for phenylketonuria

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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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Changing dietary practices in phenylketonuria.

In recent years, there has been much focus on research on non-dietary treatments in phenylketonuria (PKU). However, diet is likely to remain the major treatment for many years to come, since it has continued to be developed and consistent improvements have occurred. For example, with protein substitute, studies have tried to define the optimal dose and timing of intake; changes in palatability ...

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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...

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ژورنال

عنوان ژورنال: Cochrane Database of Systematic Reviews

سال: 2010

ISSN: 1465-1858

DOI: 10.1002/14651858.cd001304.pub2