Abstract
Hereditary angioedema (HAE) is an autosomal dominant disease associated with episodic attacks of nonpitting edema that may affect any external or mucosal body surface. Attacks most often affect the extremities, causing local swelling, the GI tract, leading to severe abdominal pain, and the mouth and throat, at times causing asphyxiation. Most patients with HAE have low levels of the plasma serine protease inhibitor C1 inhibitor. The edema in these patients is caused by unregulated generation of bradykinin. Effective chronic therapy of patients with impeded androgens or plasmin inhibitors has been available for decades, but in the United States, we do not have therapy for acute attacks. Five companies have completed or are in the process of conducting phase 3 clinical trials, double-blind, placebo-controlled studies of products designed to terminate acute attacks or to be used in prophylaxis. Two companies, Lev Pharmaceuticals and CSL Behring, have preparations of C1 inhibitor purified from plasma that have been used in Europe for decades (trade names Cinryze and Berinert P, respectively). One company, Pharming, has developed a recombinant C1 inhibitor preparation. One company, Dyax, is testing a kallikrein inhibitor (ecallantid...Continue Reading
References
May 26, 1977·The New England Journal of Medicine·H Asamoto, M Furuta
Mar 16, 1979·Deutsche medizinische Wochenschrift·K Bork, G Witzke
Dec 23, 1976·The New England Journal of Medicine·J A GelfandM M Frank
May 1, 1976·Annals of Internal Medicine·M M FrankJ P Atkinson
Apr 1, 1990·The Journal of Clinical Investigation·M CugnoM Cicardi
May 28, 1987·The New England Journal of Medicine·J AlsenzM Loos
Jan 1, 1968·International Archives of Allergy and Applied Immunology·H GewurzR A Good
Apr 28, 1973·Lancet·J T HendersonM D Sumerling
Aug 31, 1972·The New England Journal of Medicine·A L ShefferF S Rosen
Oct 1, 1969·The British Journal of Dermatology·R H Champion, P J Lachmann
Jan 1, 1980·The Journal of Allergy and Clinical Immunology·A AgostoniB Marasini
Sep 1, 1981·The Journal of Allergy and Clinical Immunology·A L ShefferK F Austen
May 1, 1982·American Journal of Diseases of Children·R Schwartz
Mar 6, 1980·The New England Journal of Medicine·J E GadekM M Frank
Jun 20, 1996·The New England Journal of Medicine·A T WaytesM M Frank
Jan 6, 1999·Journal of Hepatology·D CramponJ P Zarski
Mar 20, 2001·Archives of Internal Medicine·K Bork, S E Barnstedt
Apr 17, 2002·The Journal of Clinical Investigation·Eun D HanAlvin E Davis
May 2, 2002·Journal of Hepatology·Konrad Bork, Volker Schneiders
Jul 30, 2002·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Henriette FarkasBeáta Visy
Sep 30, 2003·Glycobiology·Kate KolesJohannis P Kamerling
Oct 15, 2003·Immunology Letters·Eun D Han LeeAlvin E Davis
Oct 24, 2003·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Marco Cicardi, Lorenza Zingale
Oct 24, 2003·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Anthony Williams, Lynn G Baird
Dec 15, 2004·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Alvin E Davis
Oct 8, 2005·The Journal of Allergy and Clinical Immunology·Martijn B A van DoornJan Nuijens
Nov 8, 2005·Transfusion·Konrad BorkJochen Hardt
Feb 8, 2006·The Journal of Allergy and Clinical Immunology·Teresa Caballero, Concepción López-Serrano
Apr 25, 2006·The Journal of Allergy and Clinical Immunology·Marcel LeviC Erik Hack
Aug 19, 2006·Expert Opinion on Investigational Drugs·Jerrold H Levy, Penrose S O'Donnell
Nov 1, 2006·Allergy·M BasT K Hoffmann
Nov 7, 2006·Immunology and Allergy Clinics of North America·Bruce L Zuraw
Dec 21, 2006·Clinical and Experimental Immunology·H J LonghurstK Khair
Jun 15, 2007·The Journal of Allergy and Clinical Immunology·Lynda SchneiderTess Schmalbach
Citations
May 1, 2012·European Journal of Pediatrics·V WahnW Kreuz
Mar 4, 2009·Rheumatology·Bilkish CassimKanti D Bhoola
Jul 16, 2008·Current Opinion in Pediatrics·Evan Bailey, Marcus Shaker
Jul 30, 2010·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Marco Cicardi, Andrea Zanichelli
Jul 30, 2010·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Henriette Farkas
Dec 15, 2011·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Amin KananiRichard Warrington
Dec 25, 2009·Drugs·Emma D Deeks
Dec 17, 2009·Nihon Jibiinkoka Gakkai kaiho·Yuka MoritaSugata Takahashi
Jul 11, 2008·Expert Opinion on Biological Therapy·Andreas Lehmann
Sep 3, 2013·The Journal of Emergency Medicine·Lawrence M Lewis
Feb 26, 2014·The Journal of Allergy and Clinical Immunology. in Practice·Marc A Riedl
Apr 4, 2013·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·H Henry LiJonathan A Bernstein
Jan 29, 2013·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·Stephen A TillesJoshua P Cohen
Apr 13, 2011·The Journal of Allergy and Clinical Immunology·Albert L ShefferWilliam E Pullman
Apr 20, 2010·La Revue de médecine interne·L Bouillet
Oct 3, 2009·The Journal of Allergy and Clinical Immunology·Fleur BossiFrancesco Tedesco
Mar 23, 2013·Clinical and Experimental Allergy : Journal of the British Society for Allergy and Clinical Immunology·Y-Y XuT J Craig
Apr 29, 2014·The Journal of Allergy and Clinical Immunology·Jonathan A BernsteinDana Wallace
Dec 17, 2014·La Presse médicale·Isabelle Boccon-Gibod
Jun 21, 2016·European Journal of Pharmacology·Mohamed M El-KadyAhmed A Soliman
Dec 31, 2009·The Journal of Rheumatology·Menachem Rottem, Reuven Mader
Nov 8, 2011·American Journal of Health-system Pharmacy : AJHP : Official Journal of the American Society of Health-System Pharmacists·Michael C Thomas, Samit Shah
Dec 2, 2009·Current Opinion in Allergy and Clinical Immunology·Efimia Papadopoulou-Alataki
Oct 1, 2008·Clinical Chemistry·Arturo BorzutzkyRaif S Geha
Nov 15, 2011·Critical Care Medicine·Anton A IgoninPaul Herscu
Dec 24, 2010·British Journal of Pharmacology·D KolteZ Shariat-Madar
Mar 16, 2013·Experimental Dermatology·Christian HünefeldMartin Röcken
Jun 14, 2012·Postgraduate Medicine·Jonathan A Bernstein, Joseph J Moellman
Aug 20, 2021·The Journal of Biological Chemistry·Charles A WarwickYuriy M Usachev