Chronic granulomatous disease presenting in a 69-year-old man.
Chronic granulomatous disease presenting in a 69-year-old man.
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一名 69 岁男性患有慢性肉芽肿病。
DOI：
10.1056/nejm199112193252506
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发表时间：
1991
期刊：
影响因子：
--
通讯作者：
Dinauer,MC
中科院分区：
文献类型：
--
作者：
Schapiro,BL;Newburger,PE;Klempner,MS;Dinauer,MC
慢性肉芽肿性疾病是一种罕见的遗传性疾病，其特征是从儿童期开始的严重复发性细菌和真菌感染。1由于吞噬细胞的NADPH氧化酶产生的超氧化物和相关微生物氧化剂不足，慢性肉芽肿性疾病患者的吞噬细胞对摄入的微生物的杀灭能力存在缺陷。活性氧化酶复合物由膜蛋白和胞质蛋白组成，它们催化NADPH到氧的单电子转移，从而形成超氧化物。2现在认识到慢性肉芽肿病可由至少四种不同氧化酶多肽的遗传缺陷引起。
CHRONIC granulomatous disease is a rare inherited disease characterized by severe recurrent bacterial and fungal infections beginning in childhood.1Phagocytes from patients with chronic granulomatous disease exhibit defective killing of ingested microorganisms because of deficient production of Superoxide and related microbial oxidants by the NADPH oxidase of the phagocyte. The active oxidase complex is composed of both membrane and cytosolic proteins that catalyze the one-electron transfer from NADPH to oxygen, thus forming Superoxide.2It is now recognized that chronic granulomatous disease can result from genetic defects in at least four different oxidase polypeptides.The most common form of chronic granulomatous . . .