Hildenhagen O, Bitter-Suermann D
Dtsch Med Wochenschr. 1985 Sep 27;110(39):1498-501. doi: 10.1055/s-2008-1069035.
Inherited deficiency of the 5th complement component (C5) was the cause of recurrent meningococcal meningitis in a woman aged 20 years. Familial investigations confirmed an autosomal co-dominant type of inheritance for the defect. Whereas additional loss of chemotaxis only exists with a genetic defect in C5 complement, bacteriolytic activity of serum is lacking in all genetic defects of the terminal complement components C5 to C8. An intact bacteriolysis, however, is necessary for resistance against Neisseria meningitidis and Neisseria gonorrhoeae. Neisseria infections, particularly recurrent meningococcal meningitis or disseminated gonococcal infections, suggest the presence of a congenital or acquired deficiency in one of the complement components C5 to C8.
一名20岁女性反复发生脑膜炎球菌性脑膜炎的病因是遗传性第五补体成分(C5)缺乏。家族调查证实该缺陷为常染色体共显性遗传类型。仅在C5补体存在基因缺陷时才会出现趋化性的额外丧失,而在终末补体成分C5至C8的所有基因缺陷中,血清的溶菌活性均缺乏。然而,完整的溶菌作用对于抵抗脑膜炎奈瑟菌和淋病奈瑟菌是必需的。奈瑟菌感染,尤其是反复发生的脑膜炎球菌性脑膜炎或播散性淋球菌感染,提示补体成分C5至C8之一存在先天性或后天性缺乏。