O'Gorman M R, Corrochano V
Department of Pediatrics, Northwestern University, Children's Memorial Hospital, Chicago, Illinois 60614.
Clin Diagn Lab Immunol. 1995 Mar;2(2):227-32. doi: 10.1128/cdli.2.2.227-232.1995.
Chronic granulomatous disease (CGD) is characterized by defective killing of intracellular microorganisms due to mutations in one of the four known components of the NADPH oxidase system. This system is responsible for the generation of superoxide and related antimicrobial oxidants. Diagnosis of CGD requires the demonstration of an abnormal oxidase system in the leukocytes of affected patients. Recently, several flow cytometry-based procedures which measure various reactive oxygen intermediates generated by the NADPH oxidase system have been developed. Most of the procedures developed to date require time-consuming granulocyte isolation, washing, and counting procedures, or they lack sensitivity. We have modified an existing procedure such that cell labelling and stimulation are performed directly in whole blood. Optimization of this procedure and its use in the diagnosis of patients with CGD or X-linked carriers are presented.
慢性肉芽肿病（CGD）的特征是，由于NADPH氧化酶系统四个已知组分之一发生突变，导致细胞内微生物杀伤功能缺陷。该系统负责生成超氧化物及相关抗微生物氧化剂。CGD的诊断需要证明受影响患者白细胞中的氧化酶系统异常。最近，已经开发出几种基于流式细胞术的方法，用于测量由NADPH氧化酶系统产生的各种活性氧中间体。迄今为止开发的大多数方法都需要耗时的粒细胞分离、洗涤和计数程序，或者缺乏敏感性。我们对现有方法进行了改进，以便直接在全血中进行细胞标记和刺激。本文介绍了该方法的优化及其在CGD患者或X连锁携带者诊断中的应用。
