Contact System Activation and Bradykinin Generation in Angioedema: Laboratory Assessment and Biomarker Utilization.
Autor: | Christiansen SC; Department of Medicine, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA 92093, USA., Zuraw BL; Department of Medicine, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA 92093, USA; Medicine Service, San Diego Veterans Administration Healthcare System, 3350 La Jolla Village Drive, San Diego, CA 92161, USA. Electronic address: bzuraw@ucsd.edu. |
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Jazyk: | angličtina |
Zdroj: | Immunology and allergy clinics of North America [Immunol Allergy Clin North Am] 2024 Aug; Vol. 44 (3), pp. 543-560. Date of Electronic Publication: 2024 May 18. |
DOI: | 10.1016/j.iac.2024.03.011 |
Abstrakt: | The role of contact system activation has been clearly established in the pathogenesis of hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH). C1 inhibitor (C1INH)-protease complexes, levels of functional C1INH, plasma kallikrein activation, and cleavage of high-molecular-weight kininogen have each been associated with disease activity. More recently, HAE with normal levels of C1INH (HAE-nl-C1INH) has been recognized. Six genetic mutations have been identified which are linked to HAE-nl-C1INH phenotypes. The majority of individuals with HAE-nl-C1INH fall into the unknown category. There is substantial evidence that bradykinin generation underlies the recurrent attacks of swelling in some of these cohorts. Competing Interests: Disclosure None. (Copyright © 2024 Elsevier Inc. All rights reserved.) |
Databáze: | MEDLINE |
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