1. Actively managed: managed with licensed drugs for prophylaxis or therapy of HAE attacks (consensus guidelines, Bowen, All., Asthma and Imm. 2001)
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  7. Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006;101(3):619-27.
  8. Bork K, Barnstedt SE. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J Am Dent Assoc 2003;134(8):1088-94.
  9. Bork K, Hardt J, Schicketanz KH, Ressel N. Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. Arch Intern Med 2003;163(10):1229-35.
  10. Bork K, Siedlecki K, Bosch S, Schopf RE, Kreuz W. Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clinic Proc 2000;75(4):349-54.
  11. Bowen T, Cicardi M, Bork K, et al. Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol 2008;100(1 Suppl 2):S30-40.
  12. Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine 1992;71(4):206-15.
  13. Bracho FA. Hereditary angioedema. Curr Op Hematol 2005;12(6):493-8.
  14. Pappalardo E, Cicardi M, Duponchel C, et al. Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. J Allergy Clin Immunol 2000;106(6):1147-54.
  15. Bork K, Barnstedt SE, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet 2000;356(9225):213-7.
  16. Binkley KE, Davis A, 3rd. Clinical, biochemical, and genetic characterization of a novel estrogendependent inherited form of angioedema. J Allergy Clin Immunol 2000;106(3):546-50.
  17. Dewald G, Bork K. Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal C1 inhibitor. Biochem Biophys Res Comm 2006;343(4):1286-9.
  18. Cichon S, Martin L, Hennies HC, et al. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet 2006;79(6):1098-104.
  19. Hall JM. Bradykinin receptors: pharmacological properties and biological roles. Pharmacol Therap 1992;56(2):131-90.
  20. Dray A. Kinins and their receptors in hyperalgesia. Can J Physiol Pharmacol 1997;75(6):704-12.
  21. Bas M, Adams V, Suvorava T, Niehues T, Hoffmann TK, Kojda G. Nonallergic angioedema: role of bradykinin. Allergy 2007;62(8):842-56.
  22. Dray A, Perkins M. Bradykinin and inflammatory pain. Trends Neurosci 1993;16(3):99-104.
  23. Kaplan AP, Joseph K, Silverberg M. Pathways for bradykinin formation and inflammatory disease. J Allergy Clin Immunol 2002;109(2):195-209. pathophysiology of angioedema. Int Immunopharmacol 2003;3(3):311-7.
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  25. Gompels MM, Lock RJ, Abinun M, et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol 2005;139(3):379-94.
  26. Note: prevalence assumption is 1:40,000. Source: Population data from Statistics Canada; pediatric not excluded.