普通变异型免疫缺陷病临床分析  

Clinical characteristics of common variable immunodeficiency disease

作　　者：林连君[1] 宋一楠[2] 王禹川[1] 张晓琳[1] 周国鹏[1] 宋以信[1] 

机构地区：[1]北京大学第一医院老年科,北京100034 [2]北京大学第一医院检验科,北京100034

出　　处：《中华医院感染学杂志》2014年第9期2221-2223,共3页Chinese Journal of Nosocomiology

基　　金：国家自然科学基金资助项目(81141001)

摘　　要：目的探讨普通变异型免疫缺陷病（CVID）的临床特点,增加临床医师对该病的认识,以减少漏诊和延迟诊断的发生。方法回顾性分析北京大学第一医院2000年1月-2013年11月确诊的5例CVID患者的临床资料、实验室检查和治疗。结果确诊CVID患者5例,起病中位年龄为5岁,确诊中位年龄为15岁,发病到确诊的中位时间为6.5年;首次就诊的主要症状是发热,常见感染包括肺炎5例、鼻窦炎及胃肠道感染各2例,急性中耳炎、角膜炎、病毒性脑炎各1例,2例患者存在支气管扩张;血清IgG、IgA和IgM的中位水平分别是2.2g/L、〈0.1g/L和0.1g/L;CD3-/CD19＋B细胞水平下降,为1.0% ～3.1%,CD3＋T细胞范围86.0% ～91.4%;所有患者均接受静脉丙种球蛋白治疗和抗炎治疗,临床症状好转。结论 CVID以反复感染为突出表现,感染部位多见于呼吸系统或消化系统;血清免疫球蛋白水平检测和淋巴细胞亚群分析对于确诊有重要价值。OBJECTIVE To explore the clinical characteristics of common variable immunodeficiency disease（CVID） and enhance the awareness of this disease so as to reduce the missing diagnosis or delayed diagnosis.METHODS The clinical data of 5patients with confirmed CVID who were treated in the Peking University First Hospital were retrospectively analyzed,including the clinical laboratory examinations and treatment.RESULTS Of the 5patients with confirmed CVID,the median age of onset of the disease was 5years old,the conformed median age was 15 years old,and the median age from the onset to the confirmation was 6.5years.The fever was the major symptom for the first diagnosis,including 5cases of pneumonia,2cases of sinusitis,2cases of gastrointestinal tract infections,1case of acute otitis media,1case of keratitis,1case of viral meningitis,and 2cases of bronchiectasis.The median level of serum IgG was 2.2g/L,the serum IgA less than 0.1g/L,the serum IgM 0.1g/L;the level of CD3-/CD19 ＋B cell was decreased by 1.0%to 3.1%,and the CD3 ＋T cell ranged between 86.0%and 91.4%.All the patients received the intravenous gamma globulin treatment and anti-inflammatory therapy,and the clinical symptoms were improved.CONCLUSION The recurrent infection is the salient manifestation of the patients with CVID,the respiratory system and digestive system are the common infection sites.It is of significant value for the diagnosis to detect the level of serum immunoglobulin and analyze the lymphocytes subsets.

关 键 词：普通变异型免疫缺陷病 感染 免疫球蛋白 淋巴细胞亚群 丙种球蛋白 

分 类 号：R593[医药卫生—内科学]