title: Hereditary angioedema with normal C1 inhibitor function: consensus of an international expert panel.
abstract: A new form of hereditary angioedema (HAE) with normal C1 inhibitor (C1INH) was first described in 2000. The lack of clear diagnostic criteria, the heterogeneity among affected patients, and the varying names given to this disease have led to substantial confusion among both physicians  and patients. This study was designed to bring more clarity to the diagnosis and potential treatment of HAE with normal C1INH. An international symposium of experts was convened to review the field and develop consensus opinions that could help clinicians who evaluate and manage these patients.  Criteria were developed for the diagnosis of HAE with normal C1INH in patients with recurrent angioedema in the absence of concurrent urticaria. In addition, potential therapeutic strategies are discussed. The consensus criteria developed during this symposium will allow physicians to better  diagnose and treat patients with HAE with normal C1INH.
authors: Department of Medicine, University of California San Diego, La Jolla, California 92093-0732, USA. bzuraw@ucsd.edu
authors: Bruce L
authors: BL
authors: Zuraw
authors: Konrad
authors: K
authors: Bork
authors: Karen E
authors: KE
authors: Binkley
authors: Aleena
authors: A
authors: Banerji
authors: Sandra C
authors: SC
authors: Christiansen
authors: Anthony
authors: A
authors: Castaldo
authors: Allen
authors: A
authors: Kaplan
authors: Marc
authors: M
authors: Riedl
authors: Charles
authors: C
authors: Kirkpatrick
authors: Markus
authors: M
authors: Magerl
authors: Christian
authors: C
authors: Drouet
authors: Marco
authors: M
authors: Cicardi
mesh_terms: D000465:Algorithms; D054179:Angioedemas, Hereditary / Q000145:classification / Q000175:diagnosis* / Q000276:immunology; D050718:Complement C1 Inhibitor Protein / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D003937:Diagnosis, Differential; D006801:Humans; D007391:International Cooperation; D017410:Practice Guidelines as Topic
chemical_list: D050718:Complement C1 Inhibitor Protein