Executive functioning of 4 children with hyperphenylalaninemia from childhood to adolescence

Hyperphenylalaninemia is a variant of phenylketonuria, and debate remains as to what, if any, active management of this condition is required to preserve cognitive function and psychological well-being. This study is the first to examine longitudinally the executive function (EF) in adolescents with...

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Published in:Pediatrics (Evanston) 2015-04, Vol.135 (4), p.e1072-e1074
Main Authors: Sharman, Rachael, Sullivan, Karen A, Jones, Toni, Young, Ross McD, McGill, Jim
Format: Article
Language:English
Subjects:
Adolescence
Adolescent
Brain
Care and treatment
Child
Child development
Child, Preschool
Childhood
Children
Cognition Disorders - diagnosis
Cognition Disorders - genetics
Company business management
Diet, Protein-Restricted
Executive Function
Female
Health aspects
Humans
Longitudinal Studies
Male
Management
Mental health
Neuropsychological Tests - statistics & numerical data
Neuropsychology
Pediatrics
Phenylketonuria
Phenylketonurias - diagnosis
Phenylketonurias - diet therapy
Phenylketonurias - genetics
Phenylketonurias - psychology
Psychometrics
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Summary:Hyperphenylalaninemia is a variant of phenylketonuria, and debate remains as to what, if any, active management of this condition is required to preserve cognitive function and psychological well-being. This study is the first to examine longitudinally the executive function (EF) in adolescents with hyperphenylalaninemia. Two sibling pairs with mild hyperphenylalaninemia underwent neuropsychological examination in early childhood and again in adolescence using EF tests that were highly sensitive to phenylalanine exposure. By early adolescence, none of the 4 children demonstrated EF impairment. The children demonstrated a typical developmental trajectory of EF from childhood to adolescence, given phenylalanine exposure consistent with their condition.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2013-4200