Journal of Developmental and Physical Disabilities

, Volume 12, Issue 4, pp 317–332

Executive Function in School-Aged Children with Phenylketonuria

  • Mary Lou Smith
  • Paula Klim
  • William B. Hanley
Article

DOI: 10.1023/A:1009480013237

Cite this article as:
Smith, M.L., Klim, P. & Hanley, W.B. Journal of Developmental and Physical Disabilities (2000) 12: 317. doi:10.1023/A:1009480013237

Abstract

The hypothesis that, by school age, children with early-treated phenylketonuria (PKU) have specific cognitive deficits in executive function skills was examined in 19 children with PKU and 19 age-, sex-, and IQ-matched control participants. Five tasks were used, three chosen to measure executive, or frontal-lobe, functions (problem-solving, working memory, and verbal fluency) and two as control measures (verbal memory and spatial perception). Children with PKU performed more poorly on the problem-solving task and on the verbal memory task, suggesting that cognitive impairments associated with PKU persist into the school-age years. In addition, cognitive performance among the children with PKU was found to be specific to the phenylalanine (phe) levels at the time of testing. The high-phe group performed consistently more poorly than the control group on four of the six measures of the problem-solving task and on the verbal memory task, whereas the low-phe group was comparable to the control group on all measures.

phenylketonuria (PKU) frontal lobe executive function 

Copyright information

© Plenum Publishing Corporation 2000

Authors and Affiliations

  • Mary Lou Smith
    • 1
    • 2
  • Paula Klim
    • 1
  • William B. Hanley
    • 3
    • 4
  1. 1.Department of PsychologyUniversity of Toronto and Hospital for Sick ChildrenTorontoCanada
  2. 2.Department of PediatricsUniversity of Toronto and Hospital for Sick ChildrenTorontoCanada
  3. 3.Department of PediatricsUniversity of Toronto and Hospital for Sick ChildrenTorontoCanada
  4. 4.Division of Clinical GeneticsUniversity of Toronto and Hospital for Sick ChildrenTorontoCanada

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