Neuropsychological speed tests and blood phenylalanine levels in patients with phenylketonuria: A meta-analysis

被引:80
作者
Albrecht, Julia [1 ]
Garbade, Sven F. [1 ]
Burgard, Peter [1 ]
机构
[1] Heidelberg Univ, Ctr Paediat & Adolescent Med, D-69120 Heidelberg, Germany
关键词
Phenylketonuria; Phenylalanine; Meta-analysis; Neuropsychological speed test; Treatment recommendations; EARLY-TREATED PHENYLKETONURIA; EVENT-RELATED POTENTIALS; SUSTAINED ATTENTION; EXECUTIVE FUNCTION; TYROSINE SUPPLEMENTATION; PREFRONTAL DYSFUNCTION; PLASMA PHENYLALANINE; BRAIN-FUNCTION; ADULT PATIENTS; CHILDREN;
D O I
10.1016/j.neubiorev.2008.11.001
中图分类号
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要
Although pathogenesis of phenylketonuria is not completely understood, a low phenylalanine diet is effective to prevent severe neurological impairment, mental retardation and behavioural difficulties. Treatment recommendations heavily rely on neuropsychological research; however, single study results are ambiguous, what is reflected in substantial variation of US, British, German, Dutch and French recommendations for blood phenylalanine concentrations for adolescents and adults. We conducted a meta-analysis estimating the influence of age, phenylalanine level, and type of neuropsychological test on effect sizes (standardized differences between controls and patients) of computer-based speed measurements in phenylketonuric patients. The effect of blood phenylalanine level on effect size was more pronounced in children and adolescents than in adults, with choice reaction time being particularly sensitive for phenylalanine concentrations. Results corroborate all recommendations for children. With the exception of the US and Dutch recommendations, all recommendations for adolescents seem to be too liberal. The same effect size is predicted for adult phenylalanine concentrations between 750 and 1500 mu mol/L not suggesting a preference for any of the published treatment recommendations for adulthood. (c) 2008 Elsevier Ltd. All rights reserved.
引用
收藏
页码:414 / 421
页数:8
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