Canadian hereditary angioedema guideline

被引：70

作者：

Betschel, Stephen ^{[1
]}

Badiou, Jacquie ^{[2
]}

Binkley, Karen ^{[1
]}

Hebert, Jacques ^{[3
]}

Kanani, Amin ^{[4
]}

Keith, Paul ^{[5
]}

Lacuesta, Gina ^{[6
]}

Yang, Bill ^{[7
]}

Aygoeren-Puersuen, Emel ^{[8
]}

Bernstein, Jonathan ^{[9
]}

Bork, Konrad ^{[10
]}

Caballero, Teresa ^{[11
]}

Cicardi, Marco ^{[12
]}

Craig, Timothy ^{[13
,14
]}

Farkas, Henriette ^{[15
]}

Longhurst, Hilary ^{[16
]}

Zuraw, Bruce ^{[17
]}

Boysen, Henrik ^{[18
]}

Borici-Mazi, Rozita ^{[19
]}

Bowen, Tom ^{[20
,21
]}

Dallas, Karen ^{[22
]}

Dean, John ^{[23
]}

Lang-Robertson, Kelly ^{[1
]}

Laramee, Benoit ^{[24
]}

Leith, Eric ^{[25
]}

Mace, Sean ^{[1
]}

McCusker, Christine ^{[26
]}

Moote, Bill ^{[27
]}

Poon, Man-Chiu ^{[28
]}

Ritchie, Bruce ^{[29
,30
]}

Stark, Donald ^{[4
]}

Sussman, Gordon ^{[1
]}

Waserman, Susan ^{[5
]}

机构：

[1] Univ Toronto, Toronto, ON, Canada

[2] HAE Canada, Notre Dame Des Lourdes, MB, Canada

[3] Univ Laval, Dept Med, Quebec City, PQ G1K 7P4, Canada

[4] Univ British Columbia, Dept Med, Vancouver, BC, Canada

[5] McMaster Univ, Dept Med, Hamilton, ON, Canada

[6] Dalhousie Univ, Dept Med, Halifax, NS, Canada

[7] Univ Ottawa, Sch Med, Ottawa, ON K1N 6N5, Canada

[8] Goethe Univ Frankfurt, D-60054 Frankfurt, Germany

[9] Univ Cincinnati, Dept Internal Med, Cincinnati, OH USA

[10] Johannes Gutenberg Univ Mainz, Univ Hosp, Dept Dermatol, D-55122 Mainz, Germany

[11] Hosp La Paz, Hlth Res Inst, Madrid, Spain

[12] Univ Milan, Osped L Sacco, Dept Internal Med, Milan, Italy

[13] Penn State Univ, Dept Med, Hershey, PA USA

[14] Penn State Univ, Dept Pediat, Hershey, PA USA

[15] Semmelweis Univ, Fac Med, Dept Internal Med 3, H-1085 Budapest, Hungary

[16] Barts & London NHS Trust, Dept Immunol, London, England

[17] Univ Calif San Diego, San Diego, CA 92103 USA

[18] HAE Int, Skanderborg, Denmark

[19] Queens Univ, Dept Med, Kingston, ON K7L 3N6, Canada

[20] Univ Calgary, Dept Med, Calgary, AB, Canada

[21] Univ Calgary, Dept Paediat, Calgary, AB T2N 1N4, Canada

[22] Saskatoon Hlth Reg, Saskatoon, SK, Canada

[23] BC Childrens Hosp, Vancouver, BC, Canada

[24] Univ Montreal, Ctr Hosp, Montreal, PQ, Canada

[25] Univ Toronto, Dept Med, Oakville, ON, Canada

[26] McGill Univ, Ctr Hlth, Dept Immunol, Montreal, PQ, Canada

[27] Western Univ, Dept Med, London, ON, Canada

[28] Southern Alberta Rare Blood & Bleeding Disorder P, Calgary, AB, Canada

[29] Univ Alberta, Dept Med, Edmonton, AB, Canada

[30] Univ Alberta, Dept Med Oncol, Edmonton, AB, Canada

来源：

ALLERGY ASTHMA AND CLINICAL IMMUNOLOGY | 2014年 / 10卷

关键词：

Hereditary angioedema; Guideline; Recommendations; Acute attacks; Short-term prophylaxis; Long-term prophylaxis; Self-administration; Individualized therapy; Quality of life; Comprehensive care; GRADE; C1 INHIBITOR DEFICIENCY; QUALITY-OF-LIFE; INTERNATIONAL WORKING GROUP; LONG-TERM SURVEY; ACUTE ATTACKS; UNITED-STATES; C1-INHIBITOR CONCENTRATE; ECALLANTIDE TREATMENT; ATTENUATED ANDROGENS; REPLACEMENT THERAPY;

D O I：

10.1186/1710-1492-10-50

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

引用

页数：18

共 87 条

[21] The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe): background and methodology [J].