@inbook{a624f1248ba448549add032d2639e28c,
title = "Brain white matter integrity mediates the relationship between phenylalanine control and executive abilities in children with phenylketonuria",
abstract = "We tested the hypothesis that brain white matter integrity mediates the relationship between phenylalanine (Phe) control and executive abilities in children with phenylketonuria (PKU; N = 36). To do so, we examined mean diffusivity (MD) from diffusion tensor imaging (DTI) in two white matter brain regions (posterior parietal–occipital, PPO; centrum semiovale, CSO) and lifetime phenylalanine (Phe) exposure; the executive abilities examined included verbal strategic processing, nonverbal strategic processing, and working memory. Mediation modeling showed that MD in the PPO and CSO mediated the relationship between Phe exposure and nonverbal strategic processing, MD in the CSO mediated the relationship between Phe exposure and verbal strategic processing, and MD in the PPO mediated the relationship between Phe exposure and working memory. These exploratory findings demonstrate the importance of using sophisticated modeling procedures to understand the interplay among metabolic control, neural factors, and functional outcomes in individuals with PKU.",
keywords = "Brain, Executive abilities, Mediation, Neuroimaging, Phenylketonuria, White matter",
author = "Anna Hood and Jerrel Rutlin and Shimony, {Joshua S.} and Grange, {Dorothy K.} and White, {Desiree A.}",
note = "Funding Information: This research was supported by the National Institute of Child Health and Human Development (R01HD044901 and U54HD087011), an Investigator Sponsored Trial grant from BioMarin Pharmaceutical Inc., the Intellectual and Developmental Disabilities Research Center at Washington University with funding from the National Institute of Child Health and Human Development (P30HD062171) and the James S. McDonnell Foundation. Funding Information: Anna Hood, Jerrel Rutlin, and Joshua Shimony declare that they have no conflict of interest. Desiree White and Dorothy Grange have received research grants from BioMarin Pharmaceutical Inc. and have served as consultants for BioMarin Pharmaceutical Inc. Publisher Copyright: {\textcopyright} SSIEM and Springer-Verlag Berlin Heidelberg 2016.",
year = "2017",
doi = "10.1007/8904_2016_579",
language = "English",
series = "JIMD Reports",
publisher = "Springer",
pages = "41--47",
booktitle = "JIMD Reports",
}