Document Detail


New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab.
MedLine Citation:
PMID:  20865644     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and renal impairment. Often HUS is triggered by Shiga-like toxin- producing ESCHERICHIA COLI. Less common is atypical HUS (aHUS), which is caused by defective complement control. aHUS is associated with mutations in genes encoding complement regulatory proteins in ~50% of patients with this syndrome. Furthermore, autoantibodies that inactivate to factor H have also been linked to the disease. Initial triggers include infections, use of endothelial-affecting drugs, malignancies, transplantation, and pregnancy. Advances in our understanding of the pathogenesis of atypical HUS suggest that complement inhibition may be used as treatment for the disease. We discuss the potential benefit of the complement inhibitor eculizumab for the treatment of aHUS.
Authors:
Ozlem Köse; Lothar-Bernd Zimmerhackl; Therese Jungraithmayr; Christoph Mache; Jens Nürnberger
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Publication Detail:
Type:  Journal Article     Date:  2010-09-23
Journal Detail:
Title:  Seminars in thrombosis and hemostasis     Volume:  36     ISSN:  1098-9064     ISO Abbreviation:  Semin. Thromb. Hemost.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-09-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0431155     Medline TA:  Semin Thromb Hemost     Country:  United States    
Other Details:
Languages:  eng     Pagination:  669-72     Citation Subset:  IM    
Copyright Information:
© Thieme Medical Publishers.
Affiliation:
Department of Nephrology, University Hospital of Essen, University Duisburg-Essen, Essen, Germany.
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