title: Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome.
abstract: To evaluate the association among complement factor H-related (CFHRs) gene deficiency, complement factor H (CFH) autoantibodies, and atypical hemolytic uremic syndrome (aHUS) susceptibility. EMBASE, PubMed, and the ISI Web of Science databases were searched for all eligible studies on the relationship amongCFHRsdeficiency, anti-FH autoantibodies, and aHUS risk. Eight case-control studies with 927 cases and 1182 controls were included in this study.CFHR1deficiency was significantly associated with an increased risk of aHUS (odds ratio (OR) = 3.61, 95% confidence interval (95% CI), 1.96, 6.63,p< 0.001), while no association was demonstrated in individuals with onlyCFHR1/R3deficiency (OR = 1.32, 95% CI, 0.50, 3.50,p= 0.56). Moreover, a more significant correlation was observed in people with both FH-anti autoantibodies andCFHR1deficiency (OR = 11.75, 95% CI, 4.53, 30.44,p< 0.001) in contrast to those with onlyCFHR1deficiency. In addition, the results were essentially consistent among subgroups stratified by study quality, ethnicity, and gene detection methods. The present meta-analysis indicated thatCFHR1deletion was significantly associated with the risk of aHUS, particularly when combined with anti-FH autoantibodies, indicating that potential interactions amongCFHR1deficiency and anti-FH autoantibodies might impact the risk of aHUS.
authors.affiliation: The First Affiliated Hospital, Xi'an Jiaotong University College of Medicine, 277 Yanta West Road, Xi'an 710061, China. jiangh2015@stu.xjtu.edu.cn.|Key Laboratory of Shaanxi Province for Craniofacial Precision Medicine Research, College of Stomatology, Xi'an Jiaotong University, Xi'an 710004, China. jiangh2015@stu.xjtu.edu.cn.|School of Public Health, Xi'an Jiaotong University Health Science Center, 76 Yanta West Road, Xi'an 710061, China. jiangh2015@stu.xjtu.edu.cn.
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
authors.affiliation: School of Public Health, Xi'an Jiaotong University Health Science Center, 76 Yanta West Road, Xi'an 710061, China. fanfan123@stu.xjtu.edu.cn.
authors.forename: Meng-Nan
authors.initials: MN
authors.lastname: Fan
authors.affiliation: School of Public Health, Xi'an Jiaotong University Health Science Center, 76 Yanta West Road, Xi'an 710061, China. yangmin1993@stu.xjtu.edu.cn.
authors.forename: Min
authors.initials: M
authors.lastname: Yang
authors.affiliation: Xi'an Honghui Hospital, 555 Friendship Road, Xi'an 710054, China. luchao0925@163.com.
authors.forename: Chao
authors.initials: C
authors.lastname: Lu
authors.affiliation: Xi'an Honghui Hospital, 555 Friendship Road, Xi'an 710054, China. xgcgfd@126.com.
authors.forename: Ming
authors.initials: M
authors.lastname: Zhang
authors.affiliation: The First Affiliated Hospital, Xi'an Jiaotong University College of Medicine, 277 Yanta West Road, Xi'an 710061, China. liuxiaoh@mail.xjtu.edu.cn.
authors.forename: Xiao-Hong
authors.initials: XH
authors.lastname: Liu
authors.affiliation: Key Laboratory of Shaanxi Province for Craniofacial Precision Medicine Research, College of Stomatology, Xi'an Jiaotong University, Xi'an 710004, China. male@mail.xjtu.edu.cn.|School of Public Health, Xi'an Jiaotong University Health Science Center, 76 Yanta West Road, Xi'an 710061, China. male@mail.xjtu.edu.cn.|Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education of China, Xi'an 710061, China. male@mail.xjtu.edu.cn.
authors.forename: Le
authors.initials: L
authors.lastname: Ma
mesh_terms: D065766:Atypical Hemolytic Uremic Syndrome / Q000453:epidemiology*; D001323:Autoantibodies; D017242:Complement Factor H / Q000172:deficiency* / Q000235:genetics / Q000276:immunology*; D000081208:Hereditary Complement Deficiency Diseases; D006801:Humans; D007674:Kidney Diseases / Q000453:epidemiology* / Q000235:genetics*; D016017:Odds Ratio; D012306:Risk; D017384:Sequence Deletion; D015432:Glomerulonephritis, Membranoproliferative
chemical_list: D001323:Autoantibodies; C493495:CFH protein, human; D017242:Complement Factor H
keywords: atypical hemolytic uremic syndrome; complement factor H; complement factor H-related genes; meta-analysis