Systematic Review and Meta-Analysis of Neuropsychiatric Symptoms and Executive Functioning in Adults With Phenylketonuria
Open Access
- 18 May 2016
- journal article
- research article
- Published by Informa UK Limited in Developmental Neuropsychology
- Vol. 41 (4), 245-260
- https://doi.org/10.1080/87565641.2016.1243109
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.Keywords
Funding Information
- BioMarin Pharmaceutical (not applicable)
This publication has 84 references indexed in Scilit:
- PRISMA-Equity 2012 Extension: Reporting Guidelines for Systematic Reviews with a Focus on Health EquityPLoS Medicine, 2012
- A latent variables examination of processing speed, response inhibition, and working memory during typical developmentJournal of Experimental Child Psychology, 2011
- High phenylalanine levels directly affect mood and sustained attention in adults with phenylketonuria: a randomised, double‐blind, placebo‐controlled, crossover trialJournal of Inherited Metabolic Disease, 2010
- Developmental differences in sustained and transient activity underlying working memoryBrain Research, 2010
- Effects of dietary management of phenylketonuria on long-term cognitive outcomeArchives of Disease in Childhood, 2007
- Prevalence, Severity, and Comorbidity of 12-Month DSM-IV Disorders in the National Comorbidity Survey ReplicationArchives of General Psychiatry, 2005
- Quantifying heterogeneity in a meta-analysisStatistics in Medicine, 2002
- Magnetic resonance imaging in phenylketonuria: Reversal of cerebral white matter changeThe Journal of Pediatrics, 1995
- Meta-analysis in clinical trialsControlled Clinical Trials, 1986
- The file drawer problem and tolerance for null results.Psychological Bulletin, 1979