RT Journal Article
SR Electronic
T1 Executive Functioning of 4 Children With Hyperphenylalaninemia From Childhood to Adolescence
JF Pediatrics
JO Pediatrics
FD American Academy of Pediatrics
SP e1072
OP e1074
DO 10.1542/peds.2013-4200
VO 135
IS 4
A1 Sharman, Rachael
A1 Sullivan, Karen A.
A1 Jones, Toni
A1 Young, Ross McD.
A1 McGill, Jim
YR 2015
UL http://pediatrics.aappublications.org/content/135/4/e1072.abstract
AB 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.