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Article
August 25, 1956

EFFECT OF A PHENYLALANINE-RESTRICTED DIET ON PATIENTS WITH PHENYLKETONURIACLINICAL OBSERVATIONS IN THREE CASES

JAMA. 1956;161(17):1628-1630. doi:10.1001/jama.1956.62970170004005b
Abstract

Some interest has been aroused recently in the possibility that patients suffering from phenylketonuria (phenylpyruvic oligophrenia), a condition in which mental deficiency is associated with an inherited abnormality in the metabolism of phenylalanine, may be benefited by giving them a diet deficient in phenylalanine. Bickel and others,1 Armstrong and Tyler,2 and Woolf and others3 all have reported improvement in the behavior of patients administered a phenylalanine-restricted diet for extended periods of time. The major biochemical abnormalities in all the patients studied were found to disappear when the high blood levels of phenylalanine, which are characteristic of the disorder, were reduced to the normal range by the dietary regimen. The nature of the mental deficiency is such that it has been difficult to determine whether there has been a significant improvement in mental functioning along with the behavioral improvement. This difficulty is readily appreciated in the assessment of

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