Progression of myocardial fibrosis by magnetic resonance imaging in patients with Duchenne and Becker muscular dystrophy
نویسندگان
چکیده
Background Duchenne (DMD) and Becker (BMD) muscular dystrophy (MD) are inherited X-linked disease characterized by progressive skeletal muscle degeneration and myocardial involvement and caused by a mutation on dystrophin gene (Xp21). Dystrophin is a sarcolemal protein that links the cytoskeleton to the basal lamina and is essential for maintenance of the muscular membrane integrity during muscular contraction. In a previous study, our group described for the first time, in a small group of 10 patients, that CMR can identify myocardial fibrosis (MF) and may be useful for detecting the early stages of cardiomyopathy in MD [1]. The evolution of the myocardial fibrosis over time in this group of patient is still unknown.
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Progression of myocardial fibrosis by magnetic resonance imaging in patients with Duchenne and Becker muscular dystrophy and preserved left ventricular ejection fraction - a randomized clinical trial for treatment with ACE inhibitors
Background Duchenne (DMD) and Becker (BMD) muscular dystrophies ( MD) are inherited X-linked diseases characterized by absence or decrease of dystrophin, a sarcolemal protein that is essential for maintenance of the muscular membrane integrity during muscular contraction. Cardiac involvement is as high, it can be clinically silent, but is often complicated by severe heart failure and high morta...
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