Loirat C, Buriot D, Peltier A P, Berche P, Aujard Y, Griscelli C, Mathieu H
Acta Paediatr Scand. 1980 Jul;69(4):553-7. doi: 10.1111/j.1651-2227.1980.tb07132.x.
A previously healthy 14-year-old boy presented with fulminant meningococcemia. He was found to have a total deficiency of C7. His serum totally lacked bactericidal activity against Neisseria meningitidis. Addition of purified C7 restored the serum hemolytic and bactericidal activity. Susceptibility to disseminated Neisseria infections has previously been reported in 3 patients with C7 deficiency, as well as in a few patients with deficiency of C5, C6 and C8. These findings emphasize the importance of intact complement mediated bactericidal activity in host defense against disseminated Neisseria infections. Evaluation of the complement system in individuals with Neisseria infections appears mandatory.
一名既往健康的14岁男孩出现暴发性脑膜炎球菌血症。发现他完全缺乏C7。其血清完全缺乏针对脑膜炎奈瑟菌的杀菌活性。添加纯化的C7可恢复血清的溶血和杀菌活性。先前已有报道称,3例C7缺乏患者以及少数C5、C6和C8缺乏患者易发生播散性奈瑟菌感染。这些发现强调了完整的补体介导的杀菌活性在宿主抵御播散性奈瑟菌感染中的重要性。对奈瑟菌感染患者的补体系统进行评估似乎是必要的。