Complete absence of the third component of complement in man.
Complete absence of the third component of complement in man.
复制标题
人类完全缺乏补体的第三种成分。
DOI：
10.1172/jci108141
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发表时间：
1975
期刊：
影响因子：
--
通讯作者：
N. Day
中科院分区：
文献类型：
--
作者：
M. Ballow;J. Shira;L. Harden;S. Yang;N. Day
一名4岁的女性患者反复感染包囊细菌和革兰氏阴性菌，发现完全缺乏总溶血性补体和C3。通过添加功能纯C3重建总溶血性补体。除了中度减少的均裂C4，所有其他C成分，均裂和径向免疫扩散测量，存在正常量。通过Ouchterlong分析，患者的血清含有C3 b灭活剂和备解素，但不含抗原C3。通过纯化的眼镜蛇毒因子（CVF）和菊粉检查交替途径的激活。这两种物质都不会导致备解素因子B活化为B。在添加部分纯化的Cordis C3时，在四种情况中的四种情况下，使用不同的Cordis C3制剂，在菊粉-血清-C3混合物中发生因子B活化为B。相比之下，在CVF-血清-C3混合物中，因子B活化为B仅发生四次中的一次。免疫粘附被认为是正常的患者的血清中，可以被去除的抗C4抗血清肼治疗。对大肠杆菌存在明显的调理素缺陷。对粗糙型大肠杆菌的血清杀菌活性。coli也有缺陷。通过Rebuck皮肤窗技术检查动员炎症反应的能力。中性粒细胞迁移延迟至第6 h。体外淋巴细胞转化和血清免疫球蛋白正常。外周血T细胞形成自发性绵羊红细胞花环的比例和B细胞通过C3受体形成EAC花环的百分比均正常。在我们的患者中缺乏C3的重要性通过被包裹的细菌的感染数量的增加和C系统的功能生物活性的降低来强调，C系统在宿主防御机制中是重要的。
A 4-yr-old female patient who has recurrent infections with encapsulated bacteria and gramnegative organisms was found to have a complete absence of total hemolytic complement and C3. Total hemolytic complement was reconstituted by the addition of functionally pure C3. With the exception of a moderately reduced homolytic C4, all other C components, measured homolytically and by radial immunodiffusion, were present in normal amounts. By Ouchterlong analysis, the patient's serum contained C3b inactivator and properdin but no antigenic C3. Activation of the alternate pathway was examined by purified cobra venom factor (CVF) and inulin. Neither of these substances led to activation of properdin factor B to B. On addition of partially purified Cordis C3, in four out of four instances and with different preparations of Cordis C3, activation of factor B to B occurred in the inulin-serum-C3 mixture. In contrast, activation of factor B to B occurred only once out of four times with CVF-serum-C3 mixtures. Immune adherence was found to be normal in the patient's serum and could be removed by anti-C4 antiserum of hydrazine treatment. A marked opsonic defect was present against Escherichia coli. Serum bactericidal activity against a rough strain of E. coli was also defective. The ability to mobilize an infalmmatory response was examined by Rebuck skin window technique. A delay in neutrophil migration occurred until the 6th h. In vitro lymphocyte transformation and serum immunoglobulins were normal. The proportion of peripheral blood T cells forming spontaneous sheep erythrocyte rosettes and the percentage of B cells forming EAC rosettes by the C3 receptor were normal. The significance of the absence of C3 in our patient is emphasized by the increased number of infections with encapsulated bacteria and the decreased functional biological activities of the C system, important in host defense mechanism(s).