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MONITORING DISEASE AND THERAPY IN DYSTROPHIN-DEFICIENT MUSCLE USING ULTRASOUND

使用超声波监测肌营养不良蛋白缺乏肌肉的疾病和治疗

基本信息

批准号：
7364380
负责人：
MICHAEL Scott HUGHES
金额：
$ 46.99万
依托单位：
WASHINGTON UNIVERSITY
依托单位国家：
美国
项目类别：
财政年份：
2009
资助国家：
美国
起止时间：
2009-07-01 至 2011-06-30
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/7364380
关键词：
10 year old 5 year old Adverse effects Animal Model Binding Biological Markers Blood Vessels Cardiac Cardiomyopathies Clinical Management Contrast Media Data Analyses Deterioration Diagnosis Disease Duchenne muscular dystrophy Dystrophin Evolution Frequencies Functional disorder Goals Heart Hereditary Disease Imaging technology Inflammatory Knowledge Left Left Ventricular Ejection Fraction Measures Methods Monitor Morphology Muscle Muscular Dystrophies Myocardial Infarction Myocardium Myopathy Oral Organ Pathologic Pathology Phenotype Physiological Process Radio Resolution Signal Transduction Skeletal Muscle Steroid therapy Steroids Structure Testing Tissues Ultrasonography Walking Work X-Linked Genetic Diseases base boys disease diagnosis imaging modality immunocytochemistry improved in vivo indexing insight mdx mouse muscle strength novel novel strategies palliative quantitative ultrasound skeletal soft tissue wasting

项目摘要

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)

这项持续工作的长期目标是通过无损成像方式对骨骼肌结构和功能进行非侵入性定量表征，并实施稳健的方法来诊断疾病和改善临床管理。特别是，我们寻求基于超声与软组织相互作用所获得的独特信息，对骨骼肌病理生理学产生新的见解。迄今为止，我们的重点是心肌梗塞和特发性心肌病的心脏重塑，以及生理和病理的血管结构和功能。我们现在建议扩展从这些努力中获得的知识，以便定量“表型”动物模型中肌营养不良蛋白缺乏导致的骨骼肌遗传性疾病的重塑过程。最终目标是生产一个用于肌营养不良症定量超声组织表征的综合包，并通过稳健的独立测量（例如力量测试）进行充分验证