Journal article
Executive Function in Phenylketonuria (PKU): Insights From the Behavior Rating Inventory of Executive Function (BRIEF) and a Large Sample of Individuals With PKU
Neuropsychology, v 34(4), pp 456-466
01 May 2020
PMID: 32202818
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Objective: Previous research has documented executive function (EF) impairments in individuals with early treated phenylketonuria (ETPKU). It remains unclear, however, whether some aspects of EF may be more affected than others. A number of factors, including small sample sizes and variability in EF tasks, have likely contributed to past mixed findings. The present objective was to elucidate further the EF profile associated with ETPKU. particularly as it relates to report-based assessment of EF. Method: Data from 286 individuals (5-48 years of age) with ETPKU on the child and adult versions of the Behavior Rating Inventory of Executive Function (BRIEF), a well-established report-based assessment tool, were analyzed. Results: The Working Memory scale showed the largest effect size in both young and older ETPKU samples, with 19% of children and 29% of adults scoring in the "abnormally elevated" range. In addition, EF impairment appeared more general (i.e., affecting more domains) in the adult sample as compared to the child sample. Exploratory analyses also suggested that the presence/absence of overall impairment on the BRIEF among our ETPKU participants could be predicted based on a small subset of items. A 10-item subset showed total classification accuracy values of 90% and above for both groups. Conclusions: Working memory represents an aspect of EF that appears to be particularly affected in individuals with ETPKU. Findings also provide preliminary support of the viability for the development and/or adoption of an abbreviated screening measure for EF difficulties in children and adults with ETPKU.
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Details
- Title
- Executive Function in Phenylketonuria (PKU): Insights From the Behavior Rating Inventory of Executive Function (BRIEF) and a Large Sample of Individuals With PKU
- Creators
- Shawn E. Christ - University of MissouriHayley E. Clocksin - University of MissouriBarbara K. Burton - Division of Genetics, Birth Defects, and Metabolism, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USMitzie L. Grant - Drexel UniversitySusan Waisbren - Boston Children's HospitalMarie-Claire Paulin - Dr. Georges-L.-Dumont University Hospital CentreDeborah A. Bilder - University of UtahDesiree A. White - Washington University in St. LouisChristopher Saville - University of Missouri
- Publication Details
- Neuropsychology, v 34(4), pp 456-466
- Publisher
- Amer Psychological Assoc
- Number of pages
- 11
- Grant note
- BioMarin Pharmaceutical, Inc.
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Psychiatry
- Web of Science ID
- WOS:000529340500007
- Scopus ID
- 2-s2.0-85084024619
- Other Identifier
- 991019167998204721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Neurosciences
- Psychology
- Psychology, Clinical