Vogler L B, Newman S L, Stroud R M, Johnston R B
Pediatrics. 1979 Oct;64(4):465-7.
A 51/2-year-old black girl with recurrent meningococcal meningitis and absence of the sixth component of complement (C6) is reported. To explore the pathogenesis of recurrent neisserial infections in C6 deficiency, a detailed analysis of her immune competence was conducted. Her serum had normal chemotactic, opsonic, alternative complement pathway, and specific antibody activity, but lacked complement-mediated bacteriolytic activity. In addition, her C6-deficient serum was indistinguishable from normal serum in a complement-dependent assay of phagocyte bactericidal activity. Absent bacteriolysis remains the only consistent defect associated with recurrent neisserial infections and absence of one of the late-acting complement components.
报告了一名5岁半的黑人女孩，患有复发性脑膜炎球菌性脑膜炎且缺乏补体第六成分（C6）。为了探究C6缺乏时奈瑟菌反复感染的发病机制，对其免疫能力进行了详细分析。她的血清具有正常的趋化、调理、替代补体途径和特异性抗体活性，但缺乏补体介导的溶菌活性。此外，在吞噬细胞杀菌活性的补体依赖性检测中，她缺乏C6的血清与正常血清无法区分。缺乏溶菌作用仍然是与奈瑟菌反复感染及一种后期补体成分缺失相关的唯一一致缺陷。
