CLINICAL TRIAL: MUSCULOSKELETAL PHENOTYPE OF MARFAN PATIENTS

这个子项目是许多利用 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 马凡氏综合征是一种常染色体显性遗传的结缔组织疾病，其特征包括主动脉根部扩张伴可能的动脉瘤和夹层、眼透镜脱位、脊柱侧凸和四肢细长和肌无力。 我们建议进行一项辅助研究，以量化基线和整个试验期间的肌肉力量、耐力、质量和质量以及骨矿物质含量和密度。 肌肉力量和耐力将通过总肌肉力量（TMS）评分进行量化，身体成分将通过人体测量DEXA进行量化。 将通过下肢MRI评估肌肉体积、信号和结构。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Marfan syndrome is an autosomal dominant connective tissue disorder which includes features such as aortic root dilatation with possible aneurysm and dissection, ocular lens dislocation, scoliosis and long, thin limbs and muscle weakness. We propose an ancillary study to quantify muscle strength, endurance, mass and quality, and bone mineral content and density at baseline and throughout this trial. Muscle strength and endurance will be quantified by a Total Muscle Strength (TMS) Score and body composition will be quantified via anthropometry DEXA. Muscle volume, signal and architecture will be assessed via MRI of the lower extremities.