Executive function in adolescents with PKU and their siblings: Associations with biochemistry

Mol Genet Metab Rep. 2015 Aug 24:4:87-8. doi: 10.1016/j.ymgmr.2015.08.001. eCollection 2015 Sep.

Abstract

Previous research shows consistent and marked executive function impairment in children with early and continuously treated phenylketonuria. This between groups analysis (phenylketonuria group vs sibling controls) found no significant differences in executive function (although adolescents with phenylketonuria performed slightly worse than their siblings). Biochemical relationships with executive function were confined to long-term measures of high phenylalanine:tyrosine ratio exposure, as well as tyrosine exposure independent of phenylalanine. This study suggests that early and continuously treated PKU results in non-significant EF differences (compared to siblings), although the influence of long-term exposure to poorer metabolic control is still evident.

Keywords: Adolescents; Dopamine; EF, executive function; Executive function; Phenylalanine:tyrosine ratio; Phenylketonuria.