MONITORING DISEASE AND THERAPY IN DYSTROPHIN-DEFICIENT MUSCLE USING ULTRASOUND

这项持续工作的长期目标是定量表征骨骼肌结构和功能的非侵入性无损成像方式，并实施强大的方法诊断疾病和改善临床管理。特别是，我们寻求开发新的见解骨骼肌病理生理学的基础上，从超声与软组织的相互作用的独特信息。在此，我们集中讨论了心肌梗死和特发性心肌病的心脏重塑，以及生理和病理血管结构和功能。现在，我们建议扩展从这些努力中获得的知识，以定量“表型”的骨骼肌的遗传疾病的重塑过程中的肌营养不良蛋白缺乏症的动物模型的结果。最终目标是产生一个全面的包，用于肌营养不良症的定量超声组织表征，该包通过强大的独立测量（例如，强度测试）

The long term goal of this continuing work is to quantitatively characterize skeletal muscle structure and function noninvasively with nondestructive imaging modalities, and to implement robust methods for diagnosing disease and improving clinical management. In particular, we seek to develop novel insights into skeletal muscle pathophysiology based on unique information available from the interaction of ultrasound with soft tissues. Heretofore we have focused on cardiac remodeling in myocardial infarction and idiopathic cardiomyopathy, and on physiologic and pathologic vascular structure and function. We now propose to extend the knowledge gained from these efforts in order to quantitatively “phenotype” the remodeling process in genetic disorders of skeletal muscle as a consequence of dystrophin deficiency in animal models. The ultimate goal is to produce a comprehensive package for quantitative ultrasound tissue characterization of muscular dystrophy, which is fully validated by robust independent measures (e.g., strength testing)