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Executive function in adolescents with PKU and their siblings: Associations with biochemistry
Journal article   Open access   Peer reviewed

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

Rachael Sharman, K Sullivan, Ross Young and J McGill
Molecular Genetics and Metabolism, Vol.4, pp.87-88
2015
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Published VersionPDF - Published Version (Open Access)CC BY V4.0 Open Access
url
https://doi.org/10.1016/j.ymgmr.2015.08.001View
Published Version

Abstract

phenylketonuria adolescents phenylalanine:tyrosine ratio executive function dopamine
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 longterm 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.

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