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)

Public Health Relevance

The Duchenne Muscular Dystrophy (DMD) is an X-linked genetic disease characterized by progressive weakness and wasting of skeletal and cardiac muscle;boys present with weakness by the age of 5 years and, if left untreated, are unable to walk without assistance by age 10 years. Therapy for DMD has been primarily palliative, with oral steroids are emerging as a first-line approach even though this treatment has serious side-effects. We propose to develop imaging technology suitable for improved diagnosis and treatment monitoring of DMD.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
1R01HL087847-01A1
Application #
7364380
Study Section
Medical Imaging Study Section (MEDI)
Program Officer
Kaltman, Jonathan R
Project Start
2009-07-01
Project End
2011-06-30
Budget Start
2009-07-01
Budget End
2010-06-30
Support Year
1
Fiscal Year
2009
Total Cost
$469,937
Indirect Cost
Name
Washington University
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
068552207
City
Saint Louis
State
MO
Country
United States
Zip Code
63130
Hughes, Michael; Marsh, Jon; Lanza, Gregory et al. (2011) Improved signal processing to detect cancer by ultrasonic molecular imaging of targeted nanoparticles. J Acoust Soc Am 129:3756-67
Hughes, Michael S; Marsh, Jon N; Agyem, Kwesi F et al. (2011) Use of smoothing splines for analysis of backscattered ultrasonic waveforms: application to monitoring of steroid treatment of dystrophic mice. IEEE Trans Ultrason Ferroelectr Freq Control 58:2361-9