播散性卡介苗感染18例分析

目的 探讨播散性卡介苗感染的临床表现、免疫学异常以及治疗转归.方法 回顾性分析2000年1月至2007年12月首都医科大学附属北京儿童医院收治的18例播散性卡介苗感染患儿的临床资料.结果 18例患儿中男13例,女5例,播散首先发生于接种部位同侧腋下淋巴结,同时播散至肺15例次,纵隔和腹腔淋巴结18例次,皮肤和软组织5例次,骨骼4例次,肝4例次,脾8例次,肾和肾上腺各1例次,脑膜1例次.18例中诊断原发免疫缺陷病12例,其中严重联合免疫缺陷病3例,慢性肉芽肿(CGD)7例,白细胞介素12/干扰素γ通路缺陷2例.18例中11例死亡,7例随访1～9年仍存活,其中4例有反复活动性皮肤和骨结核表现,3例易患反复其他病原感染.结论 播散性卡介苗感染患儿多存在原发免疫缺陷病,其中CGD和IL-12/IFN-γ通路缺陷在此类患儿中比例较高,对此类患儿应进行特殊免疫功能检查.患儿多数预后不良,应尽早明确免疫缺陷类型,并进行针对性免疫治疗.

Clinical analysjs of 18 children with disseminated Bacille Calmette-Gudrin infection

Objective To explore the clinical manifestation,immune abnormality and outcome of disseminated Bacille Calmette-Guérin(BCG) infection in children.Method The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively.Result Thirteen of the children were male among 18 patients.Disseminated infection first appeared in armpit lymph nodes ipsilateral to the vaccination site,then spread to lungs in 15,lymphnodes of mediastinum or abdomihal cavity in 18,skin and soft tissues in 5,skeletons in 4,liver in 4,spleen in 8, kidney,adrenal gland or meninges in 3.Twelve children were diagnosed to have primary immunodeficiency; 3 had severe combined immunodeficiency(SCID);7 had chronic granulomatous disease(CGD),2 had IL-12/IFN-γ passageway deficiency.Eleven of the 18 patients died,and the remaining 7 patients were followed up from 1 to 9 years and are alive at present,but presented recurrent skin and bone tuberculosis in 4 and recurrent other infection in 3.Conclusion Most Children with disseminated BCG infection had primary immunodeficiency.CGD and IL-12/IFN-γ passageway deficiency accounted for considerable proportion,so special immune function should be detected in these patients.The prognosis was poor.The type of the immunedeficiency diseases should be identified in early stage and the specific immune treatment should be siven to the patients.