Document Detail

Protease inhibitors in the treatment of hereditary angioedema.
MedLine Citation:
PMID:  14572819     Owner:  NLM     Status:  MEDLINE    
Deficiency of C1 Inhibitor leads to unopposed activation of complement, with localized, unpredictable, and sometimes life-threatening attacks of angioedema. Treatment with plasma-derived C1 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-aminocaproic 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 (Dyax) has received orphan drug status in Europe and is undergoing clinical trial in Europe and the USA.
Bruce C Ritchie
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis     Volume:  29     ISSN:  1473-0502     ISO Abbreviation:  Transfus. Apher. Sci.     Publication Date:  2003 Dec 
Date Detail:
Created Date:  2003-10-23     Completed Date:  2004-02-17     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  101095653     Medline TA:  Transfus Apher Sci     Country:  England    
Other Details:
Languages:  eng     Pagination:  259-67     Citation Subset:  T    
4-71 MSB, University of Alberta, AB, Edmonton, Canada.
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MeSH Terms
6-Aminocaproic Acid / therapeutic use
Angioedema / drug therapy*,  genetics*
Antifibrinolytic Agents / therapeutic use
Aprotinin / therapeutic use
Clinical Trials as Topic
Complement C1 Inactivator Proteins / therapeutic use
Protease Inhibitors / therapeutic use*
Tranexamic Acid / therapeutic use
Reg. No./Substance:
0/Antifibrinolytic Agents; 0/Complement C1 Inactivator Proteins; 0/Protease Inhibitors; 1197-18-8/Tranexamic Acid; 60-32-2/6-Aminocaproic Acid; 9087-70-1/Aprotinin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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