儿童重症肌无力106例

Clinical Study of 106 Myasthenia Gravis in Children

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 explore the clinical characteristics of myasthenia gravis（MG） in children. Methods One hundred and six children with MG in Children＇s Hospital of Chongqing Medical University from Apr. 2002 to Apr. 2007 were concluded in the study. The data of laboratory examinations,including virus antibody, autoantibody test,immunity function, chest X - ray or chest CT, MRI, ECG and repetitive nerve stimulation, and treatment of eholinesterase inhibitor,glucocorticoids and high -dose immunoglobulin were reviewed. Results Among 106 children, male - to - female ratio was 1 to 1.16,male 49 cases, female 57 cases. The course of disease was 3 days to 11 years. The age of onset of all 106 children with MG varied from 10 months to 14.3 years old and the mean age was （4.74 ±3.32） years old. The most common age of onset was less than 3 years old（55.7% ）. There were 41 cases newly diagnosed children and 65 cases received irregular treatment or relapsed after treatment. The ocular muscular symptoms were mostely found at the onset（84.0% ） ;brainstem type was involved at the onset （2.8%） ,generalized type was involved at the onset（ 13.2% ） ;58 out of 106 cases（54.7% ） had upper respiratory tract infection befnre the onset with MG; 41 out of 52 cases with MG had abnormal immune function or auto antibody - positive,myocardial enzymes increased in 45 ca- ses increased in varying degrees,thymoma were not found by use of sternum or CT or MRI in 69 cases,repetitive nerve stimulation of 7 out of 11 cases were abnormal; 17 cases （ 16.0% ） of the children received single pyridostigmine treatment, 10 cases （9.4%） received co -adrenal hormone and before steroid given IVIG therapy respectively were satisfied with the efficacy,and gravis symptoms were improved ;61.3% of the eases had a relapse who underwent irregular treatment or relapse after treatment,before relapse 37 eases （56.9%） received single cholines- terase inhihitor lreatment, 28 cases （43.1% ）for co - adrenal hormone, alter adrenoeortical hormone and pyridostigmine treatment. The prognosis of 72 patients（67.9% ） with MG was usually good; the causes of relapse resulted from infection （30 cases, 46.2% ） , irregular drug use （22 eases,33.8% ） , and drug withdrawal and infection （ 13 eases, 20.0% ）. Conclusions As early as age of onset,the disease easily repeated ,to be early co -adrenal hormone therapy group is the children＇s clinical features of MG.