Nigro G, Comi L I, Politano L, Limongelli F M, Nigro V, De Rimini M L, Giugliano M A, Petretta V R, Passamano L, Restucci B
Department of Clinical and Experimental Medicine, Second Naples University, Italy.
Muscle Nerve. 1995 Mar;18(3):283-91. doi: 10.1002/mus.880180304.
To evaluate the features and the course of cardiomyopathy in Becker muscular dystrophy, 68 patients--identified by clinical assessment and by reduced dystrophin labeling and/or DNA analysis--were followed in the years 1976-1993, for periods ranging from 3 to 18 years (mean 8). Patients periodically underwent clinical, electrocardiographic, echocardiographic, nuclear, and radiological assessments. Preclinical cardiac involvement was found in 67.4% of patients under 16 years of age, decreasing to 30% in patients older than 40. Clinically evident cardiomyopathy was found in 15% of patients under 16 years of age, increasing to 73% in patients older than 40. A real, dilated cardiomyopathy is the most frequent type of myocardial involvement after the age of 20. Results show that the severity of cardiac involvement can be unrelated to the severity of skeletal muscle damage and confirm that cardiac dysfunction is a primary feature of Becker muscular dystrophy.
为评估贝克型肌营养不良症中心肌病的特征及病程，1976年至1993年间，对68例经临床评估、抗肌萎缩蛋白标记降低和/或DNA分析确诊的患者进行了随访，随访时间为3至18年（平均8年）。患者定期接受临床、心电图、超声心动图、核医学及放射学评估。16岁以下患者中，67.4%存在临床前期心脏受累，40岁以上患者中该比例降至30%。16岁以下患者中，15%存在临床明显的心肌病，40岁以上患者中该比例升至73%。真正的扩张型心肌病是20岁以后最常见的心肌受累类型。结果表明，心脏受累的严重程度可能与骨骼肌损伤的严重程度无关，并证实心脏功能障碍是贝克型肌营养不良症的主要特征。
