儿童眼肌型重症肌无力的远期预后及相关因素

Long-term prognosis of ocular myasthenia gravis in children and its related factors

目的探讨儿童眼肌型重症肌无力的远期预后及影响预后的相关因素。方法选择88例发病年龄为2～14岁,初发为眼肌型重症肌无力的患儿为研究对象,随访5～10年,观察其远期预后,并采用Logistic回归模型分析其影响因素。结果 13例患者正规药物治疗2年后症状完全缓解,占14.77%;59例患者仍为眼肌型重症肌无力(包括26例症状缓解停药后复发者),占67.05%;16例患者转化为全身型重症肌无力,占18.18%,其中多数(9/16,56.25%)于发病后1年内转化为全身型重症肌无力。抗乙酰胆碱受体(AchR)抗体及胸腺异常(包括胸腺瘤和胸腺增生)与远期预后有显著相关性(P<0.05)。结论儿童重症肌无力的预后有其自身特点,由眼肌型转化为全身型的发生率较成人低,抗AchR抗体阳性和胸腺异常是转化为全身型重症肌无力的重要危险因素。

Objective To explore long-term prognosis of ocular myasthenia gravis（OMG）in children and its related factors.Methods 88 patients aged between 2 and 14 years at onset were selected for this follow-up survey,and they were primarily diagnosed as having OMG.The follow-up time was 5 years to 10 years.At the end of the follow-up,the long-term prognosis of OMG was evaluated and its related factors were analyzed by logistic regression.Results At the end of the follow-up,13 patients（14.77%）were asymptomatic after taking drugs for about two years;59 patients（67.05%）had OMG throughout the follow-up duration,including 26 recrudescent patients after drug withdrawal;and 16 patients（18.18%）converted into generalized myasthenia gravis,most of which（9/16,56.25%）occurred within 1 year after onset.The long-term prognosis of OMG had a significant correlation with the acetylcholine receptor（AchR）antibody and thymopathy,including thymoma and thymic hyperplasia（P0.05）.Conclusion Prognosis of OMG in children has some distinctive characteristics and the probability of OMG converting into generalized myasthenia gravis is lower than in adults.Several factors,such as the AchR antibody and thymopathy,may have an impact on the risk of OMG developing into generalized disease in children.