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Short‐term dietary interventions in children and adolescents with treated phenylketonuria: Effects on neuropsychological outcome of a well‐controlled population
Author(s) -
Huijbregts S. C. J.,
De Sonneville L. M. J.,
Licht R.,
Van Spronsen F. J.,
Sergeant J. A.
Publication year - 2002
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1021205713674
Subject(s) - neuropsychology , psychological intervention , phenylketonurias , medicine , pediatrics , working memory , psychology , population , phenylalanine , cognition , psychiatry , chemistry , biochemistry , amino acid , environmental health
This study addressed two questions: is there an effect of dietary interventions that induce relatively small changes in phenylalanine (Phe) concentration on neuropsychological outcome of early‐ and continuously treated phenylketonuria (PKU) patients, and are there differences in effects for PKU children and adolescents? To answer the first question, the effect of a short‐term dietary intervention (1–2 weeks) was compared for patients whose Phe concentrations increased vs those whose Phe concentrations decreased. Controls were tested twice to control for learning effects. To answer the second question, the effect of dietary interventions was examined in younger patients (aged 7–10 years) and older patients (aged 11–14 years). The effect of dietary interventions was determined with three neuropsychological tasks: one requiring sustained attention; a second demanding maintenance in working memory; and a third in which complex operations were performed in working memory. Relatively small fluctuations in Phe concentration were found to influence neuropsychological task performance of PKU patients. Patients whose Phe concentrations decreased at the second assessment showed generally more improvement than controls. Patients whose Phe concentrations had increased showed minimal improvement or deterioration of task performance. The strongest effects were observed when sustained attention and manipulation of working memory content were required. There were some indications of a greater sensitivity of younger patients to fluctuations in Phe concentration.