Dai Yi, Wei Xiaoming, Zhao Yanhuan, Ren Haitao, Lan Zhangzhang, Yang Yun, Chen Lin, Cui Liying
Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
BGI-Wuhan, Wuhan, China.
Neuromuscul Disord. 2015 Aug;25(8):617-24. doi: 10.1016/j.nmd.2015.03.002. Epub 2015 Mar 17.
Muscular dystrophies and congenital myopathies are a large group of heterogeneous inherited muscle disorders. The spectrum of muscular dystrophies and congenital myopathies extends to more than 50 diseases today, even excluding the common forms Duchenne Muscular Dystrophy, Myotonic Dystrophy and Facioscapulohumeral Dystrophy. Unfortunately, even by critical clinical evaluation and muscle pathology, diagnosis is still difficult. To potentially remediate this difficulty, we applied a microarray-based targeted next-generation sequencing (NGS) technology to diagnose these patients. There were 55 consecutive unrelated patients who underwent the test, 36 of which (65%) were found to have a causative mutation. Our result shows the accuracy and efficiency of next-generation sequencing in clinical circumstances and reflects the features and relative distribution of inherited myopathies in the Chinese population.
肌营养不良症和先天性肌病是一大类异质性遗传性肌肉疾病。如今,肌营养不良症和先天性肌病的范围已扩展至50多种疾病,甚至不包括常见的杜氏肌营养不良症、强直性肌营养不良症和面肩肱型肌营养不良症。不幸的是,即使通过严格的临床评估和肌肉病理学检查,诊断仍然困难。为了潜在地解决这一难题,我们应用了基于微阵列的靶向新一代测序(NGS)技术来诊断这些患者。有55例连续的非亲属患者接受了检测,其中36例(65%)被发现存在致病突变。我们的结果显示了新一代测序在临床情况下的准确性和效率,并反映了中国人群中遗传性肌病的特征和相对分布。