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Overactivation of Complement Alternative Pathway in Postpartum Atypical Hemolytic Uremic Syndrome Patients with Renal Involvement
Song, Di1,2,3,4; Yu, Xiao-juan1,2,3,4; Wang, Feng-mei1,2,3,4; Xu, Bing-ning5; He, Ying-dong5; Chen, Qian5; Wang, Su-xia1,2,3,4; Yu, Feng1,2,3,4; Song, Wen-chao6,7; Zhao, Ming-hui1,2,3,4,8
关键词Complement Alternative Pathway Complement Factor h Postpartum Atypical Hemolytic Uremic Syndrome Thrombotic Microangiopathies
刊名AMERICAN JOURNAL OF REPRODUCTIVE IMMUNOLOGY
2015-10-01
DOI10.1111/aji.12404
74期:4页:345-356
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Immunology ; Reproductive Biology
资助者Chinese 973 Project ; National Natural Science Foundation of China ; Chinese 973 Project ; National Natural Science Foundation of China
研究领域[WOS]Immunology ; Reproductive Biology
关键词[WOS]THROMBOTIC THROMBOCYTOPENIC PURPURA ; MEMBRANE COFACTOR PROTEIN ; GENOME-WIDE ASSOCIATION ; FACTOR-H ; LUPUS NEPHRITIS ; MACULAR DEGENERATION ; APOPTOTIC CELLS ; PLASMA-EXCHANGE ; GENE-MUTATIONS ; SYNDROME AHUS
英文摘要

Problem

Postpartum atypical hemolytic uremic syndrome (aHUS) is a life-threatening syndrome with unclear pathogenesis. The current study aimed to investigate the clinical and pathological features, complement activation status, and the genetic variations in a Chinese cohort of patients with renal biopsy-proven postpartum aHUS.

Method of study

Five patients with postpartum aHUS were recruited. Renal biopsy specimens were examined and scored. Plasma levels of complements were detected, and coding sequences of complement regulators were screened. Anti-CFH/CFI autoantibodies were further detected.

Results

Patients with postpartum aHUS patients presented with severe clinical manifestations and renal involvement. The renal biopsies of the five patients showed typical features of thrombotic microangiopathies. The levels of the following complement components, C4d, Bb, C3a, C5a, and SC5b-9, were significantly elevated in patients with postpartum aHUS compared with normal non-pregnant controls. The plasma levels of CFH and CFI significantly decreased in patients with postpartum aHUS compared with normal pregnant women. Three CFH single nucleotide polymorphisms (SNPs) were identified in the five patients. Two patients presented with CFH autoantibodies.

Conclusion

Postpartum aHUS is a clinical syndrome with severe renal damage. Genetic deficiencies and autoantibodies of CFH may lead to alternative pathway overactivation and participated in the pathogenesis of postpartum aHUS.

语种英语
所属项目编号2012CB517702 ; 81321064 ; 81470932
资助者Chinese 973 Project ; National Natural Science Foundation of China ; Chinese 973 Project ; National Natural Science Foundation of China
WOS记录号WOS:000362664200007
引用统计
被引频次:2[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/52441
专题北京大学第一临床医学院_肾脏内科
作者单位1.Peking Univ, Hosp 1, Dept Med, Div Renal, Beijing 100034, Peoples R China
2.Peking Univ, Inst Nephrol, Beijing 100034, Peoples R China
3.Minist Hlth China, Key Lab Renal Dis, Beijing, Peoples R China
4.Minist Educ China, Key Lab Chron Kidney Dis Prevent & Treatment, Beijing, Peoples R China
5.Peking Univ, Hosp 1, Dept Obstet & Gynecol, Beijing 100034, Peoples R China
6.Univ Penn, Perelman Sch Med, Inst Translat Med & Therapeut, Philadelphia, PA 19104 USA
7.Peking Tsinghua Ctr Life Sci, Beijing, Peoples R China
8.Univ Penn, Perelman Sch Med, Dept Pharmacol, Philadelphia, PA 19104 USA
推荐引用方式
GB/T 7714
Song, Di,Yu, Xiao-juan,Wang, Feng-mei,et al. Overactivation of Complement Alternative Pathway in Postpartum Atypical Hemolytic Uremic Syndrome Patients with Renal Involvement[J]. AMERICAN JOURNAL OF REPRODUCTIVE IMMUNOLOGY,2015,74(4):345-356.
APA Song, Di.,Yu, Xiao-juan.,Wang, Feng-mei.,Xu, Bing-ning.,He, Ying-dong.,...&Zhao, Ming-hui.(2015).Overactivation of Complement Alternative Pathway in Postpartum Atypical Hemolytic Uremic Syndrome Patients with Renal Involvement.AMERICAN JOURNAL OF REPRODUCTIVE IMMUNOLOGY,74(4),345-356.
MLA Song, Di,et al."Overactivation of Complement Alternative Pathway in Postpartum Atypical Hemolytic Uremic Syndrome Patients with Renal Involvement".AMERICAN JOURNAL OF REPRODUCTIVE IMMUNOLOGY 74.4(2015):345-356.
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