Nigro G, Comi L I, Limongelli F M, Giugliano M A, Politano L, Petretta V, Passamano L, Stefanelli S
Muscle Nerve. 1983 May;6(4):253-62. doi: 10.1002/mus.880060403.
Within the Campania region of southern Italy a prospective study on X-linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of death.
1969年至1980年（含）的12年间，在意大利南部的坎帕尼亚地区开展了一项关于X连锁进行性肌营养不良的前瞻性研究。杜氏肌营养不良（DMD）病例的平均发病率为每10万例男性活产21.7例，贝克肌营养不良（BMD）病例的平均发病率为每10万例男性活产3.2例。DMD患者中的家族性病例占38.5%，BMD患者中的家族性病例占50%。在DMD患者中，约6岁时出现心肌受累的病例比例很高，且逐年递增，直至生命的最后几年，95%的病例出现心脏损害。13岁之前，BMD患者中心肌受累的比例非常低，但逐渐上升，直至20岁，此时所研究病例中的80%出现心脏损害；21岁之前未发生严重心肌病。报告的数据还包括年龄对身体机能、血清肌酸激酶活性和血清肌红蛋白水平的影响、心脏损害类型以及死亡原因。
