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目的探讨儿童重症肌无力(MG)细胞和体液免疫特点。方法收集1995—2004复旦大学儿科医院神经科病房60例诊断为MG的患儿,男性34例,女性26例;发病年龄3个月至15岁。进行体液免疫检查:包括MG自身免疫抗体检查,如血清AchRab、PremRab和Titinab的测定和随访。细胞免疫检查:包括细胞因子白介素-4(IL-4)和γ-干扰素(IFN-γ)的检测。结果(1)临床分型:I型43例(72%),Ⅱ型13例(占22%,包括IIB型3例),Ⅲ型4例(6%)。(2)自身抗体检查:①初查,AchRab43.5%(20/46)阳性,PremRab41.3%(19/46)阳性;Titinab8.7%(2/23)阳性,见于AchRab阳性病例,但无胸腺瘤影像学依据。②复查,动态随访血清AchRab与PremRab,发现53.8%由阴性转变为阳性;Titinab的随访中1例阴性转变为阳性。(3)细胞因子检查:①病例组IL-4和IFN-γ的量显示较对照组差异有显著性意义;②在SPMG和对照组与SNMG和对照组的比较,IL-4未显示差异具有显著性意义,而IFN-γ显示差异具有显著性意义。结论⑴儿童MGAchRab初发病例多为阴性,MG自身抗体AchRab、PremRab、Titinab在随访中可由阴性转为阳性;且动态随访增加儿童MG中AchRab阳性率。⑵儿童MG患儿IFN-γ、IL-4表达高于对照组,表明MG患儿体内存在特异性细胞免疫活化和细胞因子网络失衡。
Objective To investigate the characteristics of childhood myasthenia gravis (MG) cells and humoral immunity. Methods A total of 60 children with MG diagnosed in Department of Neurology, Pediatric Hospital of Fudan University from 1995 to 2004 were collected. There were 34 males and 26 females. The age of onset was from 3 months to 15 years. Humoral immunity tests: including MG autoimmune antibody tests, such as serum AchRab, PremRab and Titinab determination and follow-up. Cellular immunity tests include the detection of the cytokines interleukin-4 (IL-4) and interferon-gamma (IFN-γ). Results (1) The clinical classification was type I in 43 cases (72%), type II in 13 cases (22%, including type IIB in 3 cases) and type III in 4 cases (6%). (2) Autoantibodies examination: (1) The initial examination showed that AchRab was positive in 43.5% (20/46), positive in PremRab (41.3%); positive in Titinab (8.7%), found in AchRab positive cases Athyroid tumor imaging basis. (2) The follow-up, dynamic follow-up of serum AchRab and PremRab, found that 53.8% from negative to positive; Titinab follow-up in 1 case turned negative. (3) Cytokines: (1) The amount of IL-4 and IFN-γ in the case group showed significant difference compared with the control group; ② IL-4 showed no difference between SPMG and control group and SNMG and control group Significant significance, and IFN-γ showed significant difference. CONCLUSIONS: Most cases of MGAchRab in children were mostly negative. MG autoantibodies AchRab, PremRab and Titinab turned negative from positive to negative at follow-up. The dynamic follow-up increased AchRab positive rate in children with MG. (2) The expression of IFN-γ and IL-4 in children with MG was higher than that in control group, indicating that there was specific cellular immune activation and cytokine network imbalance in MG children.