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[第八补体成分β亚基家族性缺陷导致的复发性脑膜炎]

[Recurrent meningitis in a familial defect of the beta-subunit of the 8th complement component].

作者信息

Nürnberger W, Pietsch H, Kobler P, Schwandt C, Seger R, Wahn V

机构信息

Kinderklinik B der Universität Düsseldorf.

出版信息

Dtsch Med Wochenschr. 1989 Jun 23;114(25):989-92. doi: 10.1055/s-2008-1066706.

DOI:10.1055/s-2008-1066706
PMID:2737093
Abstract

Bacterial meningitides occurred in two members of a Yugoslavian family. In one case meningitis remained a singular event, Neisseria meningitidis being identified in the CSF. The second patient developed five episodes of recurrent purulent meningitis associated with petechiae and in one instance also with arthritis of the left knee but no causative germ was found. In both patients the Western blot technique revealed a defect of the beta-subunit of the eighth component of complement that was completely eliminated by purified C8. This proved that the C8 defect was isolated and that no other component was deficient.

摘要

在一个南斯拉夫家庭的两名成员中发生了细菌性脑膜炎。其中一例脑膜炎仅为单次发病,脑脊液中鉴定出脑膜炎奈瑟菌。第二名患者出现了五次复发性化脓性脑膜炎发作,伴有瘀点,其中一次还伴有左膝关节炎,但未发现致病病菌。在两名患者中,蛋白质印迹技术均显示补体第八成分的β亚基存在缺陷,而纯化的C8可完全消除该缺陷。这证明C8缺陷是孤立存在的,不存在其他成分缺陷。

相似文献

1
[Recurrent meningitis in a familial defect of the beta-subunit of the 8th complement component].[第八补体成分β亚基家族性缺陷导致的复发性脑膜炎]
Dtsch Med Wochenschr. 1989 Jun 23;114(25):989-92. doi: 10.1055/s-2008-1066706.
2
C8 beta subunit deficiency in a patient with recurrent neisserial infections.一名复发性奈瑟菌感染患者的C8β亚基缺乏症
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Recurrent meningococcal meningitis associated with deficiencies of C8 and anti-meningococcal antibody.复发性脑膜炎球菌性脑膜炎与C8及抗脑膜炎球菌抗体缺乏相关。
J Clin Lab Immunol. 1987 May;23(1):53-6.
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[Recurrent meningococcal sepsis associated with congenital C8 deficiency: description of 2 families].[与先天性C8缺乏相关的复发性脑膜炎球菌败血症:2个家庭的描述]
Med Clin (Barc). 1983 Dec 3;81(18):789-93.
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Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review.与复发性脑膜炎球菌性脑膜炎相关的补体第八成分缺乏——病例报告及文献综述
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[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent].[一名青少年反复感染脑膜炎奈瑟菌揭示的纯合子补体B缺乏症]
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Recurrent Neisseria meningitidis bacteremia. Association with deficiency of the eighth component of complement (C8) in a Sephardic Jewish family.
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The role of complement in anti-bacterial defence.补体在抗菌防御中的作用。
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Deficiency of late complement components in patients with severe and recurrent meningococcal infections.严重复发性脑膜炎球菌感染患者晚期补体成分缺乏。
Eur J Pediatr. 1996 Aug;155(8):723-4. doi: 10.1007/BF01957164.