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2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

Tom Bowen1*, Marco Cicardi2, Henriette Farkas3, Konrad Bork4, Hilary J Longhurst5, Bruce Zuraw6, Emel Aygoeren-Pürsün7, Timothy Craig8, Karen Binkley9, Jacques Hebert10, Bruce Ritchie11, Laurence Bouillet12, Stephen Betschel9, Della Cogar1314, John Dean15, Ramachand Devaraj16, Azza Hamed17, Palinder Kamra17, Paul K Keith18, Gina Lacuesta19, Eric Leith20, Harriet Lyons1321, Sean Mace9, Barbara Mako1322, Doris Neurath23, Man-Chiu Poon24, Georges-Etienne Rivard25, Robert Schellenberg26, Dereth Rowan1321, Anne Rowe1327, Donald Stark26, Smeeksha Sur28, Ellie Tsai29, Richard Warrington30, Susan Waserman18, Rohan Ameratunga31, Jonathan Bernstein32, Janne Björkander33, Kristylea Brosz1334, John Brosz1334, Anette Bygum35, Teresa Caballero36, Mike Frank37, George Fust3, George Harmat38, Amin Kanani26, Wolfhart Kreuz7, Marcel Levi39, Henry Li40, Inmaculada Martinez-Saguer7, Dumitru Moldovan41, Istvan Nagy42, Erik W Nielsen43, Patrik Nordenfelt44, Avner Reshef45, Eva Rusicke7, Sarah Smith-Foltz46, Peter Späth47, Lilian Varga3 and Zhi Yu Xiang48

Author Affiliations

1 Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada

2 Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy

3 3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary

4 Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany

5 Department of Immunology, Barts and the London NHS Trust, London, England, UK

6 University of California, San Diego, San Diego, California, USA

7 Johann Wolfgang Goethe University, Frankfurt/Main, Germany

8 Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania, USA

9 Department of Medicine, University of Toronto, Toronto, Canada

10 Department of Medicine, Laval University, Quebec City, Quebec, Canada

11 Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, Alberta, Canada

12 Department of Medicine, CHU de Grenoble, Grenoble, France

13 Member, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH). 705 South Tower, 3031 Hospital Dr. NW, Calgary, Alberta, Canada

14 Portage La Prairie, Manitoba, Canada

15 Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada

16 Department of Medicine, Regina, Saskatchewan, Canada

17 Memorial University and Janeway Child Health Centre, St. John's, Newfoundland, Canada

18 Department of Medicine, McMaster University, Hamilton, Ontario, Canada

19 Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada

20 Department of Medicine, University of Toronto, Oakville, Ontario, Canada

21 Ancaster, Ontario, Canada

22 St. Catharines, Ontario, Canada; Member and Chair, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH

23 Transfusion Medicine, Ottawa Hospital, Ottawa, Ontario, Canada

24 Department of Medicine, University of Calgary, Calgary, Alberta, Canada

25 Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada

26 Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada

27 Halifax, Nova Scotia, Canada

28 Brampton, Ontario, Canada

29 Queen's University, Kingston, Ontario, Canada

30 Department of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada

31 University of Auckland, Auckland, New Zealand

32 Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USA

33 Department of Clinical and Experimental Medicine, County Hospital Ryhov, Jönköping, Sweden

34 Calgary, Alberta, Canada

35 Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark

36 Hospital La Paz Health Research Institute, Madrid, Spain

37 Duke University Medical Center, Durham, North Carolina, USA

38 Heim Pal Pediatric Hospital, Budapest, Hungary

39 Dept of Medicine, Academic Medical Center, Amsterdam Area, Netherlands

40 Institute for Asthma & Allergy, Wheaton and Chevy Chase, Maryland, USA

41 4th Medical Clinic, University of Medicine and Pharmacy, Tirgu Mures, Romania

42 Hungarian Association of Angioedema Patients, Budapest, Hungary

43 Nordland Hospital, Bodo, University of Tromso, Norway

44 Department of Medicine, County Hospital Ryhov, Jonkoping, Sweden

45 Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel

46 Asociación Española de Angioedema Familiar por Deficiencia del inhibidor de C1 (AEDAF), Madrid, Spain

47 Institute of Pharmacology, University of Bern, Switzerland

48 Peking Union Medical College Hospital, Beijing, China

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Allergy, Asthma & Clinical Immunology 2010, 6:24  doi:10.1186/1710-1492-6-24

Published: 28 July 2010

Abstract

Background

We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.

Objective

To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010).

Methods

The Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) http://www.haecanada.com webcite and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review.

Results

This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference.

Conclusions

Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.