The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy
The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy
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贝克尔型肌营养不良症、面肩肱型营养不良症和贝特莱姆肌病中的心脏
作者：
M. de Visser;W. D. de Voogt;G. A. la Rivière
我们报告了一项研究，评估参与的心脏在33个家族性病例贝克肌营养不良症（BMD），31个熟悉的情况下面肩肱（FSH）营养不良症，和27个家族性病例Bethlem肌病。在BMD患者中，心肌受累与年龄和肌肉骨骼受累程度相关。我们对FSH营养不良的患者进行了体格检查、胸部X线检查、心电图（EKG）和超声心动图检查。13例BMD患者（45%）的EKG变化与Duchenne型肌营养不良症相似。13名心脏病患者中只有1人需要坐轮椅。我们没有发现FSH营养不良患者心脏改变的证据。在Bethlem肌病中，只有1例患者患有肥厚型心肌病（不对称性室间隔肥大）。
We report a study, assessing involvement of the heart in 33 familial cases of Becker muscular dystrophy (BMD), 31 familiar cases of facioscapulohumeral (FSH) dystrophy, and 27 familial cases of Bethlem myopathy. In the patients with BMD, correlations of myocardial involvement with age and extent of musculoskeletal involvement were made. We performed physical examination, chest X‐ray, electrocardiographic (EKG), and echocardiographic extients with FSH dystrophy. Thirteen patients with BMD (45%) showed EKG changes similar to those found in Duchenne muscular dystrophy. Only 1 of the 13 individuals with cardiac invovement was wheelchairbound. We found no evidence of cardiac changes in the patients with FSH dystrophy. In Bethlem myopathy, only 1 patient had a form of hypertrophic cardiomyopathy (asymmetrical septal hypertrophy).