[Executive functions in children with phenylketonuria: variations as a function of phenilalanine plasm level]. / Funções executivas em crianças fenilcetonúricas: variações em relação ao nível de fenilalanina.

ABSTRACT

The present study investigates the hypothesis of a specific executive function deficit in children with Phenilketonuria (PKU) whose Phenilalanine level is between 360 and 600 mmol/l. Participants were 21 early and continuously treated 9-month-old children with PKU and 18 9-month-old controls. The children with PKU were divided into two groups on the basis of their mean phenilalanine level prior to the study the group of children whose level was between 120 and 360 mmol/l, and the group of children whose level was between 360 and 600 mmol/l. Although the three groups did not differ with regard to performance on a test of global mental development, the PKU children with high phenilalanine performed significantly worse than both the low phenilalanine PKU children and the control children on a task that assesses executive functioning.