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Dev Neuropsychol. 2016 May-Jun;41(4):245-260. Epub 2016 Nov 2.

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

Author information

1
a Department of Psychiatry , University of Utah , Salt Lake City , Utah.
2
b CTI Clinical Trial and Consulting Services Inc ., Cincinnati , Ohio.
3
c BioMarin Pharmaceutical Inc ., Novato , California.
4
d Audentes Therapeutics , San Francisco , California.

Abstract

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.

PMID:
27805419
PMCID:
PMC5152552
DOI:
10.1080/87565641.2016.1243109
[Indexed for MEDLINE]
Free PMC Article

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