播散性卡介苗病2例并文献复习.docVIP

精品文档（可编辑） 值得下载 PAGE1 / NUMPAGES1 播散性卡介苗病2例并文献复习 【摘要】 目的：探讨播散性卡介苗病的临床特点、化验室资料及治疗转归。方法：回顾性分析本院收治的2例及文献报道的41例播散性卡介苗患者的临床资料。结果：43例患儿中男28例，女15例，播散首先发生在接种部位同侧腋窝淋巴结，肺播散43例，肝、脾播散15例，皮肤播散11例，骨及骨髓播散7例，肾和肾上腺播散4例，颅内播散5例。其中5例诊断重度联合免疫缺陷病；2例诊断高IgM综合征；1例诊断高IgE综合征；16例慢性肉芽肿，其中5例基因检测存在CYBB基因突变；3例存在IL-12/IFN-γ通路缺陷。43例中死亡18例，病情迁延11例，慢性好转及病情稳定12例，失访2例。结论：播散性卡介苗病多存在原发免疫缺陷病，对此类患者应尽量行特殊免疫检查。此类疾病患儿多预后不佳，治疗方案有待进一步研究。 【关键词】 卡介苗； 接种； 播散性卡介苗病； 免疫缺陷； 随访研究 【Abstract】 Objective：To investigate the clinical features，laboratory data and treatment outcomes of disseminated BCG disease.Method：The clinical data of two patients with disseminated BCG disease in our hospital and 41 patients with disseminated BCG disease mentioned in the literature reports were retrospectively analyzed.Result：In the 43 cases of children patients，28 cases were male，15 cases were female.Dissemination first occurred in the ipsilateral axillary lymph nodes of the vaccination site，43 cases were lung spread，15 cases were liver，spleen spread，11 cases were skin spread，7 cases were bone and bone marrow disseminated，4 cases were kidney and adrenal gland spread，5 cases were intracranial spread.5 cases were diagnosed with severe combined immunodeficiency disease，2 cases were diagnosed with high IgM syndrome，1 case was diagnosed with high IgE syndrome，16 cases were diagnosed with chronic granulomatous（among which 5 cases were detected with CYBB gene mutations by genetic testing），3 cases were diagnosed with IL-12/IFN-γ pathway defects.Among the 43 cases，18 cases were death，11 cases were with protracted disease，12 cases were with chronic improvement and stable disease.2 cases were lost follow-up.Conclusion：Disseminated BCG disease is always with primary immunodeficiency disease，special immune check shall be given to these patients.Children with this disease are always with poor prognosis，its treatment needs further research. 【Key words】 Bacillus calmette guerin； Inoculate； Disseminated BCG disease； Immunodeficiency；Follow-up stu