Executive Function in School-Aged Children with Phenylketonuria
- 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
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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.