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Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome

1
The First Affiliated Hospital, Xi’an Jiaotong University College of Medicine, 277 Yanta West Road, Xi’an 710061, China
2
Key Laboratory of Shaanxi Province for Craniofacial Precision Medicine Research, College of Stomatology, Xi’an Jiaotong University, Xi’an 710004, China
3
School of Public Health, Xi’an Jiaotong University Health Science Center, 76 Yanta West Road, Xi’an 710061, China
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Xi’an Honghui Hospital, 555 Friendship Road, Xi’an 710054, China
5
Key Laboratory of Environment and Genes Related to Diseases (Xi’an Jiaotong University), Ministry of Education of China, Xi’an 710061, China
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
Academic Editor: William Chi-shing Cho
Int. J. Environ. Res. Public Health 2016, 13(12), 1209; https://doi.org/10.3390/ijerph13121209
Received: 19 September 2016 / Revised: 22 November 2016 / Accepted: 25 November 2016 / Published: 5 December 2016
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 among CFHRs deficiency, anti-FH autoantibodies, and aHUS risk. Eight case-control studies with 927 cases and 1182 controls were included in this study. CFHR1 deficiency 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 only CFHR1/R3 deficiency (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 and CFHR1 deficiency (OR = 11.75, 95% CI, 4.53, 30.44, p < 0.001) in contrast to those with only CFHR1 deficiency. In addition, the results were essentially consistent among subgroups stratified by study quality, ethnicity, and gene detection methods. The present meta-analysis indicated that CFHR1 deletion was significantly associated with the risk of aHUS, particularly when combined with anti-FH autoantibodies, indicating that potential interactions among CFHR1 deficiency and anti-FH autoantibodies might impact the risk of aHUS. View Full-Text
Keywords: complement factor H-related genes; complement factor H; atypical hemolytic uremic syndrome; meta-analysis complement factor H-related genes; complement factor H; atypical hemolytic uremic syndrome; meta-analysis
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Jiang, H.; Fan, M.-N.; Yang, M.; Lu, C.; Zhang, M.; Liu, X.-H.; Ma, L. Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome. Int. J. Environ. Res. Public Health 2016, 13, 1209.

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