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目的探讨儿童重症肌无力(MG)的临床特点。方法收集2002年4月-2007年4月重庆医科大学附属儿童医院106例MG患儿的临床资料,并对其实验室检查(包括病毒抗体、自身抗体检查、免疫功能、胸片或胸部CT、MRI、心电图及重复神经电刺激等)及胆碱酯酶抑制剂、糖皮质激素及大剂量丙种球蛋白等治疗进行回顾性分析。结果MG患儿男49例,女57例,男女比例为1:1.16,均为汉族;病程3d～11a;发病年龄10个月～14.3岁[(4.74±3.32)岁],其中3岁以下婴幼儿占55.7%。新发初诊病例41例,不规则治疗或治疗后复发病例65例;起病时表现为眼肌型占84.0%,脑干型占2.8%,全身型占13.2%;发病前有上呼吸道感染者占54.7%,52例中41例免疫功能测定异常或自身抗体阳性,45例行心肌酶谱检查均升高,69例摄胸片、CT或MRI均见胸腺瘤,11例行重复神经电刺激检查7例表现异常;17例(16.0%)患儿单用吡啶新斯的明获得满意疗效,10例(9.4%)服用激素前先予IVIG,肌无力症状有所改善;经不规则治疗或治疗后复发者占61.3%,复发前治疗方法为单用胆碱酯酶抑制剂者37例(56.9%),胆碱酯酶抑制剂和肾上腺皮质激素联合者28例(43.1%),72例(67.9%)经激素治疗,结果满意;复发患儿的诱因主要为感染30例(46.2%),不规则用药22例(33.8%),停药加感染13例(20.0%)。结论儿童MG起病年龄早,易出现病情反复,需早期合用肾上腺皮质激素治疗。
Objective To investigate the clinical features of children with myasthenia gravis (MG). Methods The clinical data of 106 children with MG were collected from April 2002 to April 2007 in Chongqing Children’s Hospital of Chongqing Medical University. Their laboratory tests (including virus antibody, autoantibodies, immune function, chest radiograph or chest CT, MRI, electrocardiogram and repeated electrical nerve stimulation, etc.) and cholinesterase inhibitors, glucocorticoids and high-dose gamma globulin treatment were retrospectively analyzed. Results There were 49 males and 57 females with a male to female ratio of 1: 1.16, all of whom were Han nationality. The course of disease ranged from 3d to 11 years. The age of onset was from 10 months to 14.3 years (4.74 ± 3.32 years) Young children accounted for 55.7%. There were 41 cases of newly diagnosed cases, 65 cases of irregular treatment or post-treatment recurrence, 84.6% of oculomotor diseases, 2.8% of brainstem types and 13.2% of systemic types on onset. Before the onset of upper respiratory tract infection Accounting for 54.7%. In 52 cases, 41 cases were abnormal in immune function or positive in autoantibodies. The myocardial enzymes were all elevated in 45 cases. Thoracic radiography in 69 cases, thymoma in CT or MRI, and repeated nerve stimulation in 11 cases Seven patients (17%, 16.0%) were treated with pyridostigmine alone, while 10 (9.4%) patients were given IVIG before hormone therapy. The symptoms of myasthenia gravis were improved. After irregular treatment or Recurrence after treatment accounted for 61.3%, 37 cases (56.9%) were treated with cholinesterase inhibitors alone, 28 cases (43.1%) were combined with cholinesterase inhibitors and adrenocorticotropic hormone, and 72 cases (67.9%) were treated with hormone. The results were satisfactory. The main causes of relapse were infection in 30 patients (46.2%), irregular medication in 22 patients (33.8%) and drug withdrawal and infection in 13 patients (20.0%). Conclusion Children with early onset of MG, prone to repeated illness, the need for early treatment with adrenal cortex hormones.