PT  - JOURNAL ARTICLE
AU  - Sharman, Rachael
AU  - Sullivan, Karen A.
AU  - Jones, Toni
AU  - Young, Ross McD.
AU  - McGill, Jim
TI  - Executive Functioning of 4 Children With Hyperphenylalaninemia From Childhood to Adolescence
AID  - 10.1542/peds.2013-4200
DP  - 2015 Apr 01
TA  - Pediatrics
PG  - e1072--e1074
VI  - 135
IP  - 4
4099  - http://pediatrics.aappublications.org/content/135/4/e1072.short
4100  - http://pediatrics.aappublications.org/content/135/4/e1072.full
SO  - Pediatrics2015 Apr 01; 135
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.