论文部分内容阅读
慢性肉芽肿症(chronic granulomatous disease,CGD)系因先天性遗传缺陷致使中性粒细胞(NPC)超氧阴离子(O_2~-)产生低下或缺如,致使其杀菌功能降低。临床表现为自婴幼儿期开始反复发生甚至是致死性的细菌或霉菌感染。近年提出了采用干扰素γ(INFγ)进行治疗,已取得良好疗效。1 NPC 的构造、生成及功能成熟的 NPC 为直径10μm～15μm的类圆形细胞,光学显微镜下可见3～4个分叶核。胞浆内含有约200个左右的颗粒,其中1/3为高电子密度的酶类及与杀菌有关的阳性荷电蛋白,包括过氧化物酶、酸性水解
Chronic granulomatous disease (CGD) is caused by the congenital genetic defects of neutrophil (NPC) superoxide anion (O 2 -) produced low or absent, resulting in reduced bactericidal function. Clinical manifestations of recurrent or even lethal bacterial or fungal infections since infancy. In recent years, the proposed treatment with interferon gamma (INFy), has achieved good results. NPC structure, generation and function Mature NPC is a circular cell with a diameter of 10μm ~ 15μm, visible under the light microscope, 3 ~ 4 lobulated. Cytoplasm contains about 200 particles, of which 1/3 for the high electron density of enzymes and bactericidal positive charge proteins, including peroxidase, acidic hydrolysis