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Simple Method To Distinguish between Primary and Secondary C3 Deficiencies

机译:区分主要和次要C3缺陷的简单方法

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Due to the increasing numbers of reported clinical cases of complement deficiency in medical centers, clinicians are now more aware of the role of the complement system in the protection against infections caused by microorganisms. Therefore, clinical laboratories are now prepared to perform a number of diagnostic tests of the complement system other than the standard 50% hemolytic component assay. Deficiencies of alternative complement pathway proteins are related to severe and recurrent infections; and the application of easy, reliable, and low-cost methods for their detection and distinction are always welcome, notably in developing countries. When activation of the alternative complement pathway is evaluated in hemolytic agarose plates, some but not all human sera cross-react to form a late linear lysis. Since the formation of this linear lysis is dependent on C3 and factor B, it is possible to use late linear lysis to routinely screen for the presence of deficiencies of alternative human complement pathway proteins such as factor B. Furthermore, since linear lysis is observed between normal human serum and primary C3-deficient serum but not between normal human serum and secondary C3-deficient serum caused by the lack of factor H or factor I, this assay may also be used to discriminate between primary and secondary C3 deficiencies.
机译:由于在医疗中心报告的补体缺乏症临床病例数量不断增加,临床医生现在更加意识到了补体系统在预防微生物引起的感染中的作用。因此,除标准的50%溶血成分测定法外,临床实验室现在准备进行补体系统的许多诊断测试。替代补体途径蛋白的缺乏与严重和反复感染有关。始终欢迎使用简便,可靠和低成本的方法进行检测和区分,特别是在发展中国家。当在溶血琼脂糖平板中评估替代补体途径的激活时,一些而非全部人类血清会发生交叉反应,形成晚期线性裂解。由于这种线性裂解的形成取决于C3和B因子,因此可以使用晚期线性裂解来常规筛查是否存在其他人类补体途径蛋白(例如B因子)的缺陷。正常人血清和原发性C3缺乏血清,但不在因缺乏H因子或I因子引起的正常人血清和次生C3缺乏血清之间,该测定法还可用于区分原发性C2和继发性C3缺乏。

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