2例慢性肉芽肿病临床特征和中性粒细胞功能测定  

CLINICAL FEATURES AND FLOW CYTOMETRY ASSAY OF 2 CHRONIC GRANULOMATOUS DISEASE

作　　者：杨晓旭[1] 农光民[2] 蒋敏[2] 

机构地区：[1]广西医科大学 [2]广西医科大学第一附属医院儿科,南宁530021

出　　处：《广西医科大学学报》2011年第3期382-385,共4页Journal of Guangxi Medical University

基　　金：广西科技攻关项目(No.桂科攻0632007-1F)

摘　　要：目的:探讨2例慢性肉芽肿病(CGD)患儿临床特点、患儿及其父母血中性粒细胞活化功能。方法:收集2005～2010年在本院临床拟诊为CGD 2例患儿的临床资料,同时采集患儿及其父母外周静脉血进行流式细胞术中性粒细胞功能测定。结果:2例皆为男性,外周血免疫细胞分型T、B细胞计数均正常,血清免疫球蛋白水平增高。流式细胞术呼吸爆发实验NAPDH氧化酶活性皆降低。结论:2例患儿临床症状符合慢性肉芽肿病改变,流式细胞术检测证实皆为中性粒细胞吞噬功能缺陷。Objective：In this study, we investigated the clinical features of 2 chronic granulomatous disease children from 2 unrelated families of Guangxi,and analyzed their the neutrophil oxidative index by flow cytometry-DHR assay. Methods： we collected the clinical data of 2 chronic granulomatous disease children who had been diagnosed clinically in 2005 and 2010, at the same time, used their peripheral blood to test neutrophil function by flow cytometry. Results：Both children were boys, T and B cell counts of peripheral blood were normal, serum immunoglohulin levels were increased. Conclusion： For 2 children, the changes of their clinical symptomses met CGD diagnosed standard, and flow eytometry test comfirded that the neutrophil function was defect.

关 键 词：慢性肉芽肿病 NADPH氧化酶 呼吸爆发 流式细胞术 

分 类 号：R725[医药卫生—儿科]