Lee T J, Snyderman R, Patterson J, Rauchbach A S, Folds J D, Yount W J
Infect Immun. 1979 Jun;24(3):656-60. doi: 10.1128/iai.24.3.656-660.1979.
The serum of a 26-year-old black man with a recent episode of meningococcemia complicated by meningitis and arthritis was found to lack hemolytic complement activity. The sixth component of complement was not detected by functional or immunochemical assays whereas other components were normal by hemolytic assay. His fresh acute-phase serum lacked complement-mediated bactericidal activity against the homologous strain of Neisseria meningitidis, but the addition of fresh normal serum or purified C6 restored bactericidal activity as well as hemolytic activity. The absence of C6 activity could not be accounted for on the basis of an inhibitor. Opsonization and chemotaxis functioned normally. Histocompatibility typing of family members did not demonstrate evidence for genetic linkage of C6 deficiency with the major histocompatibility loci. This report represents the first published case of C6 deficiency associated with bacteremic Neisseria infections in which antimeningococcal bactericidal antibodies have been definitively demonstrated against the homologous strain in the acute phase of the illness.
一名26岁黑人男性近期发生了伴有脑膜炎和关节炎的脑膜炎球菌血症,其血清被发现缺乏溶血补体活性。通过功能或免疫化学检测未检测到补体第六成分,而通过溶血检测其他成分正常。他的新鲜急性期血清缺乏针对脑膜炎奈瑟菌同源菌株的补体介导的杀菌活性,但添加新鲜正常血清或纯化的C6可恢复杀菌活性以及溶血活性。C6活性的缺失不能用抑制剂来解释。调理作用和趋化作用正常。家庭成员的组织相容性分型未显示C6缺乏与主要组织相容性位点存在遗传连锁的证据。本报告是首例发表的与菌血症性奈瑟菌感染相关的C6缺乏病例,其中在疾病急性期已明确证明存在针对同源菌株的抗脑膜炎球菌杀菌抗体。