The Dietary Treatment of 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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Megaloblastic Anaemia Complicating Dietary Treatment of Phenylketonuria in Infancy.

Nutritional deficiencies may complicate the treatment of phenylketonuria by 'synthetic' phenylalanine restricted diets. This danger is greatest in infancy when growth is rapid. We report the occurrence of megaloblastic anaemia of infancy due to folic acid deficiency, during the dietary treatment of a baby with phenylketonuria. Case Report Simon B. was born at home on July 16, 1960, the third ch...

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Intelligence and quality of dietary treatment in phenylketonuria.

The records of the Phenylketonuria Register were examined to determine the factors associated with early intellectual progress in children who had received a diet low in phenylalanine from soon after birth. A total of 1031 children were born between 1964 and 1980 and started treatment before they were 4 months of age, and 808 of them were followed up prospectively. In 263 children born between ...

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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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Treatment of classical phenylketonuria.

This paper presents some observations on the dietary treatment of phenylketonuric children seen in Glasgow during the past decade. Both the efficacy of the low phenylalanine diet in preventing brain damage (Bessman, 1966), and the adequacy of diagnostic criteria when applied in early infancy (Schneider and Garrard, 1966; Stephenson and McBean, 1967a) have recently been questioned. We therefore ...

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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 1958

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.33.167.31