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  • Title: [An adult patient with phenylketonuria before and one year after reinstitution of diet therapy].
    Author: Tiefenthaler M, Seidl R, Scheibenreiter S, Wandl-Vergesslich K, Stöckler-Ipsiroglu S.
    Journal: Wien Klin Wochenschr; 1999 Jan 15; 111(1):33-6. PubMed ID: 10067268.
    Abstract:
    BACKGROUND: In phenylketonuria (PKU) a phenylalanine restricted diet during the first years of life can prevent the development of severe cognitive damage. OBJECTIVE: Could neuropsychological or neurological changes occur in a 20-year-old patient with PKU (diagnosed early and treated until the age of 8 years) after 12 years of normal nutrition and if so, can these changes be counteracted by reinstitution of a low phenylalanine diet? METHODS: Psychological (intelligence, attention), neurophysiological (evoked potentials) and neuroradiological examination (magnetic resonance tomography [MRT] of the brain) were performed before and after one year of treatment with a diet low in phenylalanine. RESULTS: During the reinstitution of dietary therapy we observed significant improvements in attentiveness (percentage increase of 30), whereas intelligence subtests remained stable, a reduction in hyperreflexia, shortening of the latencies of the evoked potentials and a decrease in periventricular pathological signal alterations as evaluated by MRT. CONCLUSIONS: The reintroduction of a diet low in phenylalanine improved psychological and neurological symptoms in this PKU patient. We recommend a constant low-phenylalanine diet throughout life in patients with PKU.
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