MONITORING DISEASE AND THERAPY IN DYSTROPHIN-DEFICIENT MUSCLE USING ULTRASOUND

这项持续工作的长期目标是用非破坏性成像方式非侵入性地定量表征骨骼肌的结构和功能，并实施可靠的方法来诊断疾病和改善临床管理。特别是，我们寻求发展对骨骼肌病理生理学的新见解，基于从超声波与软组织的相互作用中获得的独特信息。到目前为止，我们主要关注心肌梗死和特发性心肌病的心脏重构，以及生理和病理血管结构和功能。我们现在建议扩展从这些努力中获得的知识，以便在动物模型中对由于Dstrophin缺乏而导致的骨骼肌遗传障碍的重建过程进行定量的“表型”。最终目标是为肌营养不良症的定量超声组织定征制作一套全面的包，该包通过可靠的独立测量(例如，强度测试)得到充分验证

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)