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急性链球菌感染后肾小球肾炎的补体谱

Complement profiles in acute post-streptococcal glomerulonephritis.

作者信息

Wyatt R J, Forristal J, West C D, Sugimoto S, Curd J G

机构信息

Department of Pediatrics, University of Tennessee, Memphis.

出版信息

Pediatr Nephrol. 1988 Apr;2(2):219-23. doi: 10.1007/BF00862594.

Abstract

It is well known that the hypocomplementemia of acute post-streptococcal glomerulonephritis (APSGN) is characterized by markedly reduced serum concentrations of C3 and moderately reduced levels of C5 and properdin (P). However, the extent of the activation of the classical pathway is not well defined and only limited data are available concerning serum concentrations of terminal components other than C5. In serial serum specimens from 14 children with APSGN, the presence and extent of C4 activation was directly assessed by measurement by rocket immunoelectrophoresis for C4 and C4 (C4d/C4 ratio). Elevated values for this ratio, indicating C4 activation, were found in 8 of 14 of the initial serum specimens, and in some patients the ratio remained elevated for several weeks. In contrast, the serum C4 level was low in only 1 specimen (the specimen with the highest C4d/C4 ratio). However, in 10 patients C4 concentrations within the normal range rose in serial serum specimens. Serum C2 concentrations were depressed in the initial specimens from 5 patients. The concentrations of 13 other complement component and control proteins were also measured in these specimens. Levels of terminal components, other than C5, in the initial serum specimens were normal except for depressed C8 in 3 of 13 patients and depressed C6 in 1 of 14. Of these 4 individuals, 3 had the lowest C3 levels in the study. It is concluded that the classical complement pathway is frequently activated in patients with APSGN early in the condition and that subtle abnormalities in C6 and C8 levels occasionally occur.

摘要

众所周知,急性链球菌感染后肾小球肾炎(APSGN)的低补体血症表现为血清C3浓度显著降低,C5和备解素(P)水平中度降低。然而,经典途径的激活程度尚不明确,关于C5以外的终末成分血清浓度的可用数据有限。在14例APSGN患儿的系列血清标本中,通过火箭免疫电泳法检测C4和C4d/C4比值,直接评估C4激活的存在和程度。在14份初始血清标本中的8份中发现该比值升高,表明C4激活,并且在一些患者中该比值持续升高数周。相比之下,仅1份标本(C4d/C4比值最高的标本)的血清C4水平较低。然而,在10例患者中,系列血清标本中C4浓度在正常范围内升高。5例患者初始标本中的血清C2浓度降低。还测量了这些标本中其他13种补体成分和对照蛋白的浓度。除13例患者中有3例C8降低和14例患者中有1例C6降低外,初始血清标本中C5以外的终末成分水平均正常。在这4例个体中,3例在研究中C3水平最低。得出的结论是,APSGN患者在疾病早期经典补体途径经常被激活,并且偶尔会出现C6和C8水平的细微异常。

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