Systematic Review and Meta-Analysis of Neuropsychiatric Symptoms and Executive Functioning in Adults With Phenylketonuria

被引：104

作者：

Bilder, Deborah A. ^{[1
]}

Noel, J. Kay ^{[2
]}

Baker, Erin R. ^{[2
]}

Irish, William ^{[2
]}

Chen, Yinpu ^{[3
]}

Merilainen, Markus J. ^{[3
]}

Prasad, Suyash ^{[4
]}

Winslow, Barbara J. ^{[3
]}

机构：

[1] Univ Utah, Dept Psychiat, 650 South Komas Dr,Suite 200, Salt Lake City, UT 84108 USA

[2] CTI Clin Trial & Consulting Serv Inc, Cincinnati, OH USA

[3] BioMarin Pharmaceut Inc, Novato, CA USA

[4] Audentes Therapeut, San Francisco, CA USA

来源：

DEVELOPMENTAL NEUROPSYCHOLOGY | 2016年 / 41卷 / 04期

关键词：

EARLY-TREATED PHENYLKETONURIA; PHENYLALANINE-RESTRICTED DIET; PREVIOUSLY UNTREATED PHENYLKETONURIA; MAGNETIC-RESONANCE-SPECTROSCOPY; WHITE-MATTER ABNORMALITIES; QUALITY-OF-LIFE; FOLLOW-UP; SUSTAINED ATTENTION; YOUNG-ADULTS; PKU PATIENTS;

D O I：

10.1080/87565641.2016.1243109

中图分类号：

B844 [发展心理学（人类心理学）];

学科分类号：

040202 ;

摘要：

This systematic review and meta-analysis (MA) investigates the impact of elevated blood phenylalanine (Phe) on neuropsychiatric symptoms in adults with phenylketonuria (PKU). The meta-analysis of PKU is challenging because high-quality evidence is lacking due to the limited number of affected individuals and few placebo-controlled, double-blind studies of adults with high and low blood Phe. Neuropsychiatric symptoms associated with PKU exceed general population estimates for inattention, hyperactivity, depression, and anxiety. High Phe is associated with an increased prevalence of neuropsychiatric symptoms and executive functioning deficits whereas low Phe is associated with improved neurological performance. Findings support lifelong maintenance of low blood Phe.

引用

页码：245 / 260

页数：16

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