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Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population

Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population
Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population
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.
0141-8955
419-430
Huijbregts, S.C.J.
1268dc4d-9c81-4452-9da6-82cdc9dd39f8
de Sonneville, L.M.J.
38de127a-43ac-48f9-a0a5-95e5ad2baa5d
Licht, R.
f00f825e-915f-4306-a530-413c43765897
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Sergeant, J.A.
2f434c2e-8247-4721-b4b0-b0d1849c843f
Huijbregts, S.C.J.
1268dc4d-9c81-4452-9da6-82cdc9dd39f8
de Sonneville, L.M.J.
38de127a-43ac-48f9-a0a5-95e5ad2baa5d
Licht, R.
f00f825e-915f-4306-a530-413c43765897
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Sergeant, J.A.
2f434c2e-8247-4721-b4b0-b0d1849c843f

Huijbregts, S.C.J., de Sonneville, L.M.J., Licht, R., van Spronsen, F.J. and Sergeant, J.A. (2002) Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population. Journal of Inherited Metabolic Disease, 25 (6), 419-430. (doi:10.1023/A:1021205713674).

Record type: Article

Abstract

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.

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Published date: 2002

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Local EPrints ID: 18576
URI: http://eprints.soton.ac.uk/id/eprint/18576
ISSN: 0141-8955
PURE UUID: 502d9db5-427f-4049-a6fc-912d3f0e16ae

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Date deposited: 02 Dec 2005
Last modified: 15 Jul 2019 19:28

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Contributors

Author: S.C.J. Huijbregts
Author: L.M.J. de Sonneville
Author: R. Licht
Author: F.J. van Spronsen
Author: J.A. Sergeant

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