Browsing by Author "Kaslow, Jacob A."

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    Leveraging Cardiac Magnetic Resonance Imaging to Assess Skeletal Muscle Progression in Duchenne Muscular Dystrophy
    (Elsevier, 2022) Kaslow, Jacob A.; Sokolow, Andrew G.; Donnelly, Thomas; Buchowski, Maciej S.; Damon, Bruce M.; Markham, Larry W.; Burnette, W. Bryan; Soslow, Jonathan H.; Pediatrics, School of Medicine
    Duchenne Muscular Dystrophy (DMD) is characterized by muscle deterioration and progressive weakness. As a result, patients with DMD have significant cardiopulmonary morbidity and mortality that worsens with age and loss of ambulation. Since most validated muscle assessments require ambulation, new functional measures of DMD progression are needed. Despite several evaluation methods available for monitoring disease progression, the relationship between these measures is unknown. We sought to assess the correlation between imaging metrics obtained from cardiac magnetic resonance imaging (CMR) and functional assessments including quantitative muscle testing (QMT), spirometry, and accelerometry. Forty-nine patients with DMD were enrolled and underwent CMR, accelerometry and QMT at baseline, 1-year and 2-year clinic visits with temporally associated pulmonary function testing obtained from the medical record. Imaging of the upper extremity musculature (triceps and biceps) demonstrated the most robust correlations with accelerometry (p<0.03), QMT (p<0.02) and spirometry (p<0.01). T1-mapping of serratus anterior muscle showed a similar, but slightly weaker relationship with accelerometry and QMT. T2-mapping of serratus anterior demonstrated weak indirect correlation with aspects of accelerometry. These images are either routinely obtained in standard CMR or can be added to a protocol and may allow for a more comprehensive assessment of a patient’s disease progression.
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    Spirometry Correlates with Physical Activity in Patients with Duchenne Muscular Dystrophy
    (Wiley, 2023) Kaslow, Jacob A.; Sokolow, Andrew G.; Donnelly, Thomas; Buchowski, Maciej S.; Markham, Larry W.; Burnette, W. Bryan; Soslow, Jonathan. H.; Pediatrics, School of Medicine
    Background: Duchenne muscular dystrophy (DMD) is associated with progressive muscle weakness and respiratory decline. To date, studies have focused on respiratory decline and loss of ambulation as a metric of loss of skeletal muscle strength. However, new functional measures can assess skeletal muscle disease regardless of ambulatory status. The relationship between these tests and concurrent lung function is currently unexplored. Objective: To assess the correlation between spirometry measurements and functional muscle assessments such as accelerometry and quantitative muscle testing (QMT). Methods: Enrolled patients with DMD underwent accelerometry and QMT at study clinic visits. Any pulmonary function testing within 6 months of visit was obtained from the electronic medical record. The Spearman correlation coefficient was used to assess the relationship between spirometry and functional muscle testing. Results: Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1 ) demonstrated the strongest correlation with accelerometry. Both FVC and FEV1 showed a similar relationship to accelerometry when activity was divided into intensity categories, with low intensity and moderate-to-vigorous activity categories showing the strongest correlation. Maximal expiratory pressure (MEP) and FVC showed the most robust correlations with total QMT (p < 0.001 and p < 0.01, respectively). Conclusion: Lung function, specifically FVC percent predicted and FEV1 %p, shows a good correlation with upper and lower extremity skeletal muscle functional testing such as accelerometry and QMT.