期刊
SEMINARS IN THROMBOSIS AND HEMOSTASIS
卷 36, 期 6, 页码 669-672出版社
THIEME MEDICAL PUBL INC
DOI: 10.1055/s-0030-1262889
关键词
Atypical hemolytic uremic syndrome; complement activation; complement inhibitor; eculizumab
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 similar to 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.
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