Dynamics of complement activation in aHUS and how to monitor eculizumab therapy-Reference-Cited by-同舟云学术

Dynamics of complement activation in aHUS and how to monitor eculizumab therapy

Published:2014-09-11 Issue:11 Volume:124 Page:1715-1726
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Noris Marina¹,Galbusera Miriam¹,Gastoldi Sara¹,Macor Paolo²,Banterla Federica¹,Bresin Elena¹,Tripodo Claudio³,Bettoni Serena¹,Donadelli Roberta¹,Valoti Elisabetta¹,Tedesco Francesco⁴,Amore Alessandro⁵,Coppo Rosanna⁵,Ruggenenti Piero⁶,Gotti Eliana⁶,Remuzzi Giuseppe¹⁶

Affiliation:

1. IRCCS - Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,” Ranica, Bergamo, Italy and “Centro Anna Maria Astori” Science and Technology Park Kilometro Rosso, Bergamo, Italy;

2. Department of Life Sciences, University of Trieste, Trieste, Italy;

3. Tumor Immunology Unit, Human Pathology Section, Department of Health Sciences, University of Palermo, Palermo, Italy;

4. IRCCS, Istituto Auxologico Italiano, Milan, Italy;

5. Unit of Nephrology, Dialysis and Transplantation, Regina Margherita University Hospital, Turin, Italy; and

6. Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy

Abstract

Key Points Endothelial-restricted complement activation occurs in aHUS, and clinical remission relies on efficient endothelial complement inhibition. Ex vivo serum-induced endothelial C5b-9 deposits are a sensitive tool to monitor complement activation and eculizumab effectiveness in aHUS.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/124/11/1715/1468410/1715.pdf

Reference37 articles.

1. Atypical hemolytic-uremic syndrome.;Noris;N Engl J Med,2009

2. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.;Noris;Clin J Am Soc Nephrol,2010

3. Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome.;Goicoechea de Jorge;Proc Natl Acad Sci USA,2007

4. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome.;Frémeaux-Bacchi;Blood,2008

5. Thrombomodulin mutations in atypical hemolytic-uremic syndrome.;Delvaeye;N Engl J Med,2009

Cited by 283 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. Case report: Timing of eculizumab treatment in catastrophic antiphospholipid syndrome;Frontiers in Immunology;2024-09-10

2. Characterizing the association between complement-mediated TMA and cognitive dysfunction using MRI and neurocognitive assessment;Blood Vessels, Thrombosis & Hemostasis;2024-09

3. Exuberant Endothelial C5b-9 Formation in Recurrent and De Novo Posttransplant Thrombotic Microangiopathy;Kidney International Reports;2024-08

4. Urinary sC5b-9 is Better Linked to Albuminuria Than to Intrarenal Inflammation in Common Kidney Disease;Kidney International Reports;2024-08

5. Ex vivo C5b-9 Deposition Test to Monitor Complement Activity in Clinical and Subclinical Atypical Hemolytic Uremic Syndrome and in Transplantation-Associated Thrombotic Microangiopathy;Kidney International Reports;2024-07