文章摘要
肖君 ，张函 ，何丹 ，谢敏杰 ，王伟.儿童期重症肌无力临床特点及免疫指标变化[J].神经损伤功能重建,2018,13(6):289-291
儿童期重症肌无力临床特点及免疫指标变化
Study on Clinical Features and Immunological Changes of Childhood-onset Myasthenia Gravis
  
DOI：
中文关键词: 儿童期重症肌无力  IgG  IgA  IgM  C3  C4  IgG亚型
英文关键词: childhood-onset myasthenia gravis  IgG  IgA  IgM  C3  C4  IgG subclasses
基金项目:
作者单位
肖君1 ，张函1 ，何丹2 ，谢敏杰1 ，王伟1 1.华中科技大学同 济医学院附属同济 医院神经内科 2.中山大学附属第 一医院神经内科 
摘要点击次数: 2867
全文下载次数: 3017
中文摘要:
      目的：对儿童期重症肌无力（MG）患者的临床特点及免疫指标变化进行研究。方法：儿童期MG患者 70例纳入病例组，健康儿童100例纳入对照组，收集病例组患儿的发病年龄、性别、MG分型、合并症及胸腺 情况，归纳儿童期MG患者的临床特点；采集2组的外周血，检测病例组AChR抗体阳性率，检测2组血清的 免疫指标（IgG、IgA、IgM，C3、C4），定量抗体芯片技术分析2组血清中IgG亚型。结果：本组儿童期MG患者 中，约半数在5岁以前发病，眼肌型占大多数（92.9%），20.0%患者合并甲状腺功能异常，14.3%的患者合并胸 腺病变，AChR抗体阳性率67.1%。与对照组比较，病例组外周血血清IgG、IgA、IgM、C3及C4水平显著降低 （P＜0.05）；在血清IgG亚型中，IgG1和IgG3水平高于对照组（P＜0.05），其余亚型差异无统计学意义（P＞ 0.05）。结论：儿童期MG发病年龄在5岁之前及单纯眼肌型的比例占多数，自身抗体阳性率高，AChR抗体 以IgG1和IgG3为主，免疫球蛋白及补体水平明显减低。
英文摘要:
      To study the clinical features and immunological changes in patients of childhood-onset myasthenia gravis (CMG). Methods: Seventy randomly selected CMG patients and 100 age-matched healthy controls were recruited. Clinical data of the CMG group such as onset age, MG classification, gender, comorbidities, and thymus abnormalities were collected to summarize the clinical features of CMG. Peripheral blood was collected from subjects in both groups. The CMG group serum AChRAb levels were detected. Immunological testing (IgG, IgA, IgM, C3, C4) was performed on both groups. The concentrations of IgG subclasses in both groups were quantified using Quantibody Human Ig Isotype Array. Results: Of the CGM patients in this study, approximately half had an onset age before 5 years old, and a majority presented with ocular type MG (92.9% ). Comorbidities of thyroid abnormality were present in 20.0% of patients, and comorbidities of thymic abnormality were present in 14.3% of patients; 67.1% of patients were positive for AChRAb. Compared with the controls, the concentrations of serum IgG, IgA, IgM, C3, and C4 in CMG patients were significantly reduced (P<0.05). Among the IgG subclasses, IgG1 and IgG3 concentrations were higher in CMG patients than in healthy controls (P<0.05); the difference in other IgG subclasses was not significant (P> 0.05). Conclusion: Our data show that a majority of CMG patients develop the disorder before 5 years of age, present with ocular type MG, and show a high positive rate of autoantibodies. Our results suggest that AChRAb is mainly composed of IgG1 and IgG3 and that CMG is characterized by decreased levels of immunoglobulins and complements
查看全文   查看/发表评论  下载PDF阅读器
关闭