ABSTRACT
Phenylketonuria (PKU) is a hereditary disorder characterized by disrupted phenylalanine metabolism and cognitive impairment. However, the precise nature and developmental trajectory of this cognitive impairment remains unclear. The present study used a verbal fluency task to dissociate executive and verbal processes in children with PKU (n = 23; 7–18 years) and controls (n = 44; 7–19 years). Data were collected at three longitudinal timepoints over a three-year period, and the contributions of age, group, and their interaction to fluency performance were evaluated. Results indicated impairments in executive processes in children with PKU, which were exacerbated by declining metabolic control.
Acknowledgments
The authors wish to thank those who participated in our study for their contributions. We also thank Suzin Blankenship, Christen Bass, and Laurie Sprietsma for their contributions to study management, as well as the physicians, faculty, and staff of Washington University and Oregon Health & Science University who generously contributed to recruitment and phenylalanine monitoring.
Supplementary data
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