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遗传性C5缺乏症中的复发性脑膜炎球菌性脑膜炎

[Recurring meningococcal meningitis in hereditary C 5 deficiency].

作者信息

Hildenhagen O, Bitter-Suermann D

出版信息

Dtsch Med Wochenschr. 1985 Sep 27;110(39):1498-501. doi: 10.1055/s-2008-1069035.

DOI:10.1055/s-2008-1069035
PMID:3928316
Abstract

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.

摘要

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。

相似文献

1
[Recurring meningococcal meningitis in hereditary C 5 deficiency].遗传性C5缺乏症中的复发性脑膜炎球菌性脑膜炎
Dtsch Med Wochenschr. 1985 Sep 27;110(39):1498-501. doi: 10.1055/s-2008-1069035.
2
Meningococcal meningitis in familial deficiency of the fifth component of complement.补体第五成分家族性缺陷中的脑膜炎球菌性脑膜炎
Pediatrics. 1981 Jun;67(6):882-6.
3
[2 families with meningococcal infection and a hereditary disorder of the 5th component of the complement system].[两例伴有脑膜炎球菌感染及补体系统第五成分遗传性疾病的家族]
Ned Tijdschr Geneeskd. 1989 Sep 9;133(36):1796-800.
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Recurrent bacterial meningitis in patients with genetic defects of terminal complement components.终末补体成分基因缺陷患者的复发性细菌性脑膜炎
Clin Exp Immunol. 1980 Apr;40(1):16-24.
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[Complement protein hereditary deficits during purulent meningitis: study of 61 adult Tunisian patients].[化脓性脑膜炎期间补体蛋白遗传性缺陷:对61名突尼斯成年患者的研究]
Arch Inst Pasteur Tunis. 2006;83(1-4):25-34.
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[Hereditary complement C5 deficiency: study of 3 Tunisian adult cases and literature review].[遗传性补体C5缺乏症:3例突尼斯成年病例研究及文献综述]
Tunis Med. 2010 Apr;88(4):269-76.
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[Recurrent Neisseria meningitidis meningitis associated with homozygote complement C7 fraction deficiency].[复发性脑膜炎奈瑟菌脑膜炎与纯合子补体C7成分缺乏相关]
Rev Neurol (Paris). 1992;148(3):237-8.
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[The association between deficiency of terminal complement components and the occurrence of meningococcal meningitis].[终末补体成分缺乏与脑膜炎球菌性脑膜炎发生之间的关联]
Fukuoka Igaku Zasshi. 1992 May;83(5):201-8.
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Recurrent meningococcal sepsis in a presumptive immunocompetent host shown to be complement C5 deficient-a case report.疑似免疫功能正常的宿主反复发生脑膜炎奈瑟菌败血症,证实为补体 C5 缺乏症-病例报告。
APMIS. 2011 Jul;119(7):479-84. doi: 10.1111/j.1600-0463.2011.02740.x. Epub 2011 Apr 8.
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[Complement deficiencies and meningococcal disease in The Netherlands].[荷兰的补体缺陷与脑膜炎球菌病]
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引用本文的文献

1
[Recurrent meningitis in familial deficiency of the 8th component of the complement system].[补体系统第8成分家族性缺陷中的复发性脑膜炎]
Klin Wochenschr. 1987 Apr 15;65(8):387-90. doi: 10.1007/BF01745581.