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]

Unregulated kallikrein activation of bradykinin may cause hereditary angioedema:[2]

  • 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).[3][4]

References

  1. Anonymous (2024), Hereditary angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. 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.
  3. 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.
  4. Angioedema, hereditary. Online Mendelian Inheritance in Man. Retrieved on 2008-06-16.