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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.

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。

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