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家族性婴儿重症肌无力(FIMG)是一种非自身免疫性疾病。其特征是:肌无力发生在同胞兄弟(姐妹),母亲没有肌无力,出生时严重的呼吸和喂奶困难,无严重眼肌瘫痪。文献提示该病系突触前乙酰胆硷的再合成或动员不足。作者报导了3个病例。例1 26岁,女性,出生后不久出现喘息性呼吸,气管切开维持达一年之久。9岁起爬楼、跑步及举臂均困难,右眼睑轻度下垂。16岁时,因妊娠病情又复恶化,腾喜龙试验阳性,此时开始接受吡啶斯的明治疗。至妊娠第9个月时,无力加重,伴呼吸抑制,经气管切开在辅助呼吸期间分娩一健康婴儿。三周后作了胸腺切除,此后肌无力改善几周。一年后又用强的松治疗,仅第一周有轻度改善。17
Familial Infantile Myasthenia Gravis (FIMG) is a non-autoimmune disease. It is characterized by: myasthenia gravis occurs in siblings (sisters), the mother is not muscle weakness, severe breathing and feeding difficulties at birth, no serious ocular muscle paralysis. The literature suggests that the disease is pre-synaptic acetylcholine resynthesis or insufficient mobilization. The authors reported 3 cases. Example 1 A 26-year-old female, wheezing shortly after birth with tracheotomy maintained for up to a year. Climbing from 9 years old, running and arms are difficult, the right eyelid drooping lightly. 16 years old, due to deterioration of pregnancy again deteriorated, Teng Sik Lung test was positive, then began to accept pyridostigmine treatment. Until the 9th month of gestation, weakened, with respiratory depression, and tracheotomy delivered a healthy baby during assisted breathing. Thymus resection was performed after three weeks, after which muscle weakness was improved for several weeks. One year later with prednisone treatment, only a slight improvement in the first week. 17