Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Köse, O; Zimmerhackl, LB; Jungraithmayr, T; Mache, C; Nürnberger, J.
New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab.
Semin Thromb Hemost. 2010; 36(6):669-672
Doi: 10.1055/s-0030-1262889
(- Case Report)
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Mache Christoph
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- Abstract:
- 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. © Thieme Medical Publishers.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Antibodies, Monoclonal - immunology
- Antibodies, Monoclonal - therapeutic use
- Antibodies, Monoclonal, Humanized -
- Complement C3b Inactivator Proteins - genetics
- Complement C5 - immunology
- Complement Factor H - genetics
- Complement Pathway, Alternative - drug effects
- Complement Pathway, Alternative - immunology
- Female -
- Gene Deletion -
- Hemolytic-Uremic Syndrome - drug therapy
- Hemolytic-Uremic Syndrome - genetics
- Hemolytic-Uremic Syndrome - immunology
- Humans -
- Mutation -
- Polymorphism, Genetic -
- Treatment Outcome -
- Young Adult -
- Find related publications in this database (Keywords)
- Atypical hemolytic uremic syndrome
- complement activation
- complement inhibitor
- eculizumab