Hereditary angioedema
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Hereditary angioedema is an "inherited disorder that is characterized by subcutaneous and submucosal edema in the upper respiratory tract and gastrointestinal tract."[1]
Hereditary angioedema types I and II are due to insufficient activity of complement C1 inhibitor protein (C1 esterase inhibitor).
Hereditary angioedema type III may due to increased activity of coagulation factor XII (Hageman factor).[2][3]
These abnormalities lead to unregulated kallikrein activation of bradykinin.[4]
References
- ↑ Anonymous (2024), Hereditary angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ Cichon S, Martin L, Hennies HC, et al (December 2006). "Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III". Am. J. Hum. Genet. 79 (6): 1098–104. DOI:10.1086/509899. PMID 17186468. PMC 1698720. Research Blogging.
- ↑ Angioedema, hereditary. Online Mendelian Inheritance in Man. Retrieved on 2008-06-16.
- ↑ Schneider L, Lumry W, Vegh A, Williams AH, Schmalbach T (August 2007). "Critical role of kallikrein in hereditary angioedema pathogenesis: a clinical trial of ecallantide, a novel kallikrein inhibitor". J. Allergy Clin. Immunol. 120 (2): 416–22. DOI:10.1016/j.jaci.2007.04.028. PMID 17559913. Research Blogging.