一名成年脑膜炎球菌病患者合并家族性C7和C4B缺乏症。
Combined familial C7 and C4B deficiency in an adult with meningococcal disease.
作者信息
Chapel H M, Peto T E, Luzzi G A, Thompson R A, Fielder A H, Batchelor J R
出版信息
Clin Exp Immunol. 1987 Jan;67(1):55-8.
Abstract
A case of meningococcal septicaemia is reported in an adult with a deficiency of the seventh component of complement combined with a deficiency of the B locus product of C4. A family study demonstrated that the two deficiencies were not linked. This is the first time that the individual alleles of C4 were determined in a patient with a deficiency of one component of the terminal pathway. It is possible that the heterogeneous clinical picture of a terminal pathway deficiency may, in part, be explained by the co-existence of other subtle complement defects.
摘要
报告了一例成年脑膜炎球菌败血症患者，其补体第七成分缺乏并伴有C4的B位点产物缺乏。一项家族研究表明这两种缺乏症并无关联。这是首次在终末途径某一成分缺乏的患者中确定C4的各个等位基因。终末途径缺乏症的异质性临床表现可能部分是由其他细微的补体缺陷共存所导致的。
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本文引用的文献
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