|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|
|WOS标题词||Science & Technology|
|类目[WOS]||Immunology ; Reproductive Biology|
|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|
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.
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.
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.
|项目编号||2012CB517702 ; 81321064 ; 81470932|
|资助机构||Chinese 973 Project ; National Natural Science Foundation of China|
|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
|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.|