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Tract-based evaluation of white matter damage in individuals with early-treated phenylketonuria.

Abstract：

:Previous research has documented white matter abnormalities in the brains of individuals with early-treated phenylketonuria (ETPKU). The majority of these past studies have relied on a region-based approach which focused on a limited number of spatially-defined regions within the brain. In the present study, we used diffusion tensor imaging (DTI) in conjunction with tract-based spatial statistics (TBSS) to perform an extensive examination of white matter tracts in the brains of ten individuals with ETPKU (mean age = 23.2 years) and 12 healthy non-PKU individuals (mean age = 23.5 years). Consistent with past research, we found that mean diffusivity (MD) was significantly restricted in the ETPKU group, and fractional anisotropy (FA) was comparable between the ETPKU and non-PKU groups. Moreover, we found restricted axial diffusivity (AD) and radial diffusivity (RD) in our ETPKU in numerous white matter tracts, suggesting widespread white matter compromise in ETPKU. In addition, this white matter pathology was more evident in older ETPKU participants with higher blood phenylalanine (phe) levels as compared to younger participants with lower phe levels.

journal_name

J Inherit Metab Dis

journal_title

Journal of inherited metabolic disease

authors

Peng H,Peck D,White DA,Christ SE

doi

10.1007/s10545-013-9650-y

subject

Has Abstract

pub_date

2014-03-01 00:00:00

pages

237-43

issue

2

eissn

0141-8955

issn

1573-2665

journal_volume

37

pub_type

杂志文章

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