期刊
TRANSFUSION AND APHERESIS SCIENCE
卷 29, 期 3, 页码 259-267出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.transci.2003.08.004
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Deficiency of C1 Inhibitor leads to unopposed activation of complement, with localized, unpredictable, and sometimes life-threatening attacks of angioedema. Treatment with plasma-derived Cl Inhibitor rapidly aborts attacks, and may be lifesaving, but is expensive, requires use of a pooled blood product, may need to be repeated and may not be effective in autoantibody mediated angioedema. The antifibrinolytic agents aprotinin, tranexamic acid, and epsilon-amino-caproic acid are useful for prophylaxis and treatment of angioedema, likely by inhibiting plasmin. Specific drugs to replace the deficient C1 Inh have not been reported. The kallikrein inhibitor DX-88(R) (Dyax) has received orphan drug status in Europe and is undergoing clinical trial in Europe and the USA. (C) 2003 Elsevier Ltd. All rights reserved.
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