Researcher(s)
- Maxwell Moen, Human Physiology, University of Delaware
Faculty Mentor(s)
- Melissa Witman, Kinesiology and Applied Physiology, University of Delaware
Abstract
Exploring the Disease Progression of Duchenne Muscle Dystrophy (DMD) and Becker Muscle Dystrophy (BMD) Through Non-Invasive Endothelial Function Testing
Maxwell C. Moen, Alexs A. Matias, Evan C. Ciecko, Raina Lakhani, Jane Diehl, Melissa A. Witman
University of Delaware, Newark, DE
Duchenne and Becker muscle dystrophy (DMD and BMD respectively) are severe genetic disorders causing progressive muscle degeneration, leading to early declines in cardiovascular function and high fatality rates. Endothelial function is an independent predictor of cardiovascular (CV) function and has previously been reported to be attenuated in patients with DMD and BMD. However, the extent and timing of cardiovascular decline in these patients is relatively unknown.
PURPOSE: The purpose is to determine if a decline in endothelial function, assessed by reactive hyperemia index (RHI), can be detected over a period of a year for DMD and BMD patients. It was hypothesized that RHI would be lower 12 months following baseline assessments.
METHODS: Eighteen boys (7-21 yrs) diagnosed with DMD and BMD were recruited. Microvascular endothelial function was assessed using RHI derived from the EndoPAT device, a non-invasive test that utilizes probes around both index fingers and assesses the hyperemic response following 5 minutes of arterial occlusion. The RHI score is calculated by formulating a ratio between the post-deflation pulse wave amplitude and the baseline amplitude in the occluded arm, and divided by the same ratio in the non-occluded arm.
RESULTS: There was no difference between baseline and 12 month RHI values in either DMD (1.32 ± 0.433 vs 1.34 ± 0.47, p = 0.64) or BMD (2.67 ± 1.28 vs 1.845 ± 0.85, p = 0.734).
CONCLUSIONS: Changes in RHI in boys with DMD and BMD were not evident over one year’s time. Further testing over a longer duration of time is necessary to provide a wider range of progression.