Bushby K M, Thambyayah M, Gardner-Medwin D
Department of Neurobiology and Genetics, University of Newcastle upon Tyne, UK.
Lancet. 1991 Apr 27;337(8748):1022-4. doi: 10.1016/0140-6736(91)92671-n.
We measured the prevalence and incidence of Becker muscular dystrophy in the Northern Health Region of England, UK. Patients were identified from the records of the Regional Neurological Centre and Muscular Dystrophy Group laboratories, Newcastle upon Tyne, and by writing to local doctors. We used cDNA probes and/or dystrophin immunolabelling of muscle-biopsy samples to prove the diagnosis of all cases. Results were compared with the known prevalence and incidence of Duchenne muscular dystrophy. 73 patients alive and resident in the Northern Health Region were identified, giving a prevalence rate of 2.38/100,000. This compares with a prevalence of Duchenne muscular dystrophy of 2.48/100,000. The cumulative birth incidence of Becker muscular dystrophy (at least 1 in 18 450 male live births) was about one third that of Duchenne muscular dystrophy (1 in 5618 male live births), suggesting that the disorder is more common than previously thought.
我们对英国英格兰北部健康区域的贝克肌营养不良症的患病率和发病率进行了测量。通过泰恩河畔纽卡斯尔地区神经学中心和肌营养不良症研究小组实验室的记录，并向当地医生发函，来确定患者。我们使用cDNA探针和/或对肌肉活检样本进行肌营养不良蛋白免疫标记，以确诊所有病例。将结果与已知的杜氏肌营养不良症的患病率和发病率进行比较。确定了73名居住在北部健康区域且仍在世的患者，患病率为2.38/10万。相比之下，杜氏肌营养不良症的患病率为2.48/10万。贝克肌营养不良症的累积出生发病率（至少为每18450例男性活产中有1例）约为杜氏肌营养不良症（每5618例男性活产中有1例）的三分之一，这表明该疾病比之前认为的更为常见。
