Executive functioning in children and adolescents with phenylketonuria

This study addresses the effects of dietary adherence, phenylalanine (phe) levels, and age on performance of executive functioning (EF) tasks in children and adolescents with phenylketonuria (PKU). We herein collate formerly discrete findings to understand the relationship among actual clinical para...

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Bibliographic Details
Published in:	Clinical genetics Vol. 72; no. 1; pp. 13 - 18
Main Authors:	VanZutphen, KH, Packman, W, Sporri, L, Needham, MC, Morgan, C, Weisiger, K, Packman, S
Format:	Journal Article
Language:	English
Published:	Oxford, UK Blackwell Publishing Ltd 01-07-2007 Blackwell
Subjects:	Adolescent adolescents Adult Aminoacid disorders Biological and medical sciences Child children Cognition Concept Formation dietary adherence Errors of metabolism executive functioning Female Fundamental and applied biological sciences. Psychology General aspects. Genetic counseling Genetics of eukaryotes. Biological and molecular evolution Humans Intelligence Male Medical genetics Medical sciences Metabolic diseases Molecular and cellular biology Neuropsychological Tests Phenylalanine - blood phenylketonuria Phenylketonurias - blood Phenylketonurias - diet therapy Phenylketonurias - psychology Problem Solving Verbal Behavior Human Nervous system diseases Executive function Phenylketonuria adolescents Metabolic diseases Enzymopathy Adhesion Genetic disease Feeding dietary adherence Diet therapy Diet children Adolescent Genetics executive functioning Child Aminoacid disorder
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Summary:	This study addresses the effects of dietary adherence, phenylalanine (phe) levels, and age on performance of executive functioning (EF) tasks in children and adolescents with phenylketonuria (PKU). We herein collate formerly discrete findings to understand the relationship among actual clinical parameters and EF in PKU. Fifteen subjects (age range 8–20 years) with PKU were compared with the normative sample on the Delis–Kaplan EF Battery and on the Wechsler Abbreviated Scales of Intelligence to examine the relationship between EF skills, phe levels, age, and dietary adherence. At the time of the assessment, the mean age of participants was 14.8 years, mean lifetime phe levels ranged from 216 to 1200 μM (mean 594 μM); and concurrent phe levels ranged from 222 to 1730 μM (mean 660 μM). Children and adolescents with PKU showed lower performance in several EF skills: initiation of problem solving, concept formation, and reasoning. Performance on EF tasks requiring inhibitory control, cognitive flexibility and set shifting decreased at higher phe levels. Phe levels were positively correlated to age and inversely related to dietary adherence. We conclude that dynamic clinical parameters appear to govern EF in patients with PKU. We suggest that when adolescents decrease dietary compliance, changes in EF skills occur. Therefore, there is a need to specifically monitor EF skills in patients with PKU during the transition to, and during, adolescence.
Bibliography:	ark:/67375/WNG-3ZN3RHQ0-L istex:0F70A6E5846B65C8EF8599F14B537CF11C9EA536 ArticleID:CGE816 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0009-9163 1399-0004
DOI:	10.1111/j.1399-0004.2007.00816.x