Magnetic Resonance Imaging and Spectroscopy Assessment of Lower Extremity Skeletal Muscles in Boys with Duchenne Muscular Dystrophy: A Multicenter Cross Sectional Study
Magnetic Resonance Imaging and Spectroscopy Assessment of Lower Extremity Skeletal Muscles in Boys with Duchenne Muscular Dystrophy: A Multicenter Cross Sectional Study
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DOI：
10.1371/journal.pone.0106435
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发表时间：
2014-09-09
期刊：
影响因子：
3.7
通讯作者：
Vandenborne, Krista
中科院分区：
文献类型：
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作者：
Forbes, Sean C.;Willcocks, Rebecca J.;Vandenborne, Krista
简介：杜氏肌营养不良症（DMD）是一种X连锁隐性遗传疾病，导致功能缺陷。然而，这些功能下降通常无法在DMD的临床试验中量化，直到7岁之后。在这项研究中，我们假设使用H-1-MRS和MRI-T-2获得的（H2OT 2）-H-1在年轻时对肌肉受累敏感与对照组相比，DMD受试者的大队列中的炎症和肌肉损伤增加。在三个机构（佛罗里达大学、俄勒冈州健康与科学大学和费城儿童医院）使用3-特斯拉MRI仪器对31名儿童（年龄5-14岁;平均8.6 SD 2.2岁）和31名健康对照（年龄9.7 SD 2.3岁）进行MRI检查。采用多层面自旋回波（SE）轴位T2加权成像和单体素H-1-MRS测量小腿和大腿的脂质分数和（H_2 OT_2）-H-1。结果：MRI-T-2、（H_2 OT_2）-H-1和脂质分数均大于（P < 0.01）。
Introduction: Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder that results in functional deficits. However, these functional declines are often not able to be quantified in clinical trials for DMD until after age 7. In this study, we hypothesized that (H2OT2)-H-1 derived using H-1-MRS and MRI-T-2 will be sensitive to muscle involvement at a young age (5-7 years) consistent with increased inflammation and muscle damage in a large cohort of DMD subjects compared to controls.Methods: MR data were acquired from 123 boys with DMD (ages 5-14 years; mean 8.6 SD 2.2 years) and 31 healthy controls (age 9.7 SD 2.3 years) using 3-Tesla MRI instruments at three institutions (University of Florida, Oregon Health & Science University, and Children's Hospital of Philadelphia). T-2-weighted multi-slice spin echo (SE) axial images and single voxel H-1-MRS were acquired from the lower leg and thigh to measure lipid fraction and (H2OT2)-H-1.Results: MRI-T-2, (H2OT2)-H-1, and lipid fraction were greater (p