Inherited complement component deficiencies in membranoproliferative glomerulonephritis.
Inherited complement component deficiencies in membranoproliferative glomerulonephritis.
复制标题
膜增生性肾小球肾炎的遗传性补体成分缺乏。
DOI：
10.1038/ki.1983.211
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发表时间：
1983
影响因子：
19.6
通讯作者：
West,CD
中科院分区：
文献类型：
--
作者：
Coleman,TH;Forristal,J;Kosaka,T;West,CD
增殖膜遗传性补体成分缺乏症。活动性肾小球肾炎。本文对178例肾小球肾炎患者和163例正常人血清中7种补体成分的水平进行了系统的研究。44例I型和III型膜增生性肾小球肾炎患者的肾功能缺陷发生率(22.7%)显著高于正常人(6.7%，P<；0.002)和其他肾小球肾炎患者(5.2%，P<；0.001)。MPGN的成分缺陷有9例为部分缺陷，1例为次全缺陷。它们不能归因于获得性低补体血症或肾病综合征。它们长期存在，在家庭成员中发现，涉及C2、C3、B因子、C6、C7和C8。六个可能是结构基因缺失的结果，两个与结构异常成分有关，两个原因不明。结果表明，补体成分的部分缺乏是MPGN的一个易感因素。
Inherited complement component deficiencies in membranoprolifera. tive glomerulonephritis. Anecdotal reports of complement component deficiencies in patients with immune complex disease led to a systemat-ic study of the levels of seven complement components in serum specimens from 178 patients with glomerulonephritis and 163 normal subjects. Deficiencies were found with significantly higher frequency (22.7%) among 44 patients with membranoproliferative glomerulonephritis (MPGN) types I and III, than among the normal subjects (6.7%, P< 0.002) or among 134 patients with other glomerulonephritides (5.2%, P< 0.001). The component deficiencies in MPGN were partial in nine patients and subtotal in one. They could not be ascribed to acquired hypocomplementemia or to a nephrotic syndrome. They were present over long periods, were found in family members, and involved C2, C3, factor B, C6, C7, and C8. Six were presumably the result of null structural genes, two were associated with a structurally abnormal component, and two were of unknown cause. The results give evidence that partial deficiency of one or more complement components is a factor predisposing to MPGN.