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自1983年以来诊治的2100例重症肌无力(MG)病例中,发现有37例患者呈先天性发病,来自9个家系。对这些呈家系发病患者的临床、免疫学及家系特征进行了研究。结果表明,这类患者的临床及免疫学特征不同于其他类型的MG,其遗传方式符合常染色体遗传,并发现2个家系呈典型显性遗传。认为这类先天性重症肌无力不属于自身免疫性疾病,而是由其他原因所致的神经肌肉接头传递障碍性疾病,遗传因素在发病中起决定作用
Of the 2,100 cases of myasthenia gravis (MG) diagnosed and treated since 1983, 37 patients were found to have congenital disease from 9 pedigrees. The clinical, immunological and pedigree characteristics of these patients with familial onset were studied. The results showed that the clinical and immunological characteristics of these patients were different from other types of MG, the genetic method consistent with autosomal genetic, and found that two families were typical dominance. That such congenital myasthenia gravis is not an autoimmune disease, but by other causes of neuromuscular junction transmission disorder, genetic factors play a decisive role in the pathogenesis