Hereditary angioedema (hereditary C1 esterase inhibitor deficiency) is an "inherited disorder that is characterized by subcutaneous and submucosal edema in the upper respiratory tract and gastrointestinal tract."
- 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).
Hereditary deficiency is characterized by the absence of urticaria and by normal levels of complement C1q and complement C1 inhibitor protein function. complement C1 inhibitor protein antigen is low in type I and normal in type II.
- Anonymous (2015), Hereditary angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- 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.
- Hereditary angioedema. (Online Mendelian Inheritance in Man, OMIM®. Johns Hopkins University, Baltimore, MD. MIM Number: 106100. World Wide Web URL: http://omim.org/.)
- Hereditary angioedema, type III. (Online Mendelian Inheritance in Man, OMIM®. Johns Hopkins University, Baltimore, MD. MIM Number: 610618. World Wide Web URL: http://omim.org/.)
- 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.
- Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). "Acquired C1 esterase inhibitor deficiency". Ann. Intern. Med. 132 (2): 144–50. PMID 10644276.
- Maeda S, Miyawaki T, Nomura S, Yagi T, Shimada M (2003). "Management of oral surgery in patients with hereditary or acquired angioedemas: review and case report.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 96 (5): 540-3. DOI:10.1016/S1079210403005018. PMID 14600687. Research Blogging.