This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of hereditary angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients 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. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary angioedema results in 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, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. 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 and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists 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.

中文翻译：

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国际/加拿大遗传性血管性水肿指南。


这是 2014 年加拿大遗传性血管水肿指南的更新，范围扩大到包括全球遗传性血管水肿 (HAE) 患者的管理。这是加拿大和国际 HAE 专家以及加拿大遗传性血管水肿网络领导的患者团体的合作成果。本指南的目的是使用 GRADE 系统为 HAE 患者的管理提供基于证据的建议。这包括发作的治疗、短期预防、长期预防以及自我给药、个体化治疗、生活质量和综合护理的建议。该指南 2019 版的新增部分涵盖了 C1-INH 正常的 HAE 患者的诊断和急性治疗推荐治疗，以及有关孕妇和儿童患者、患者协会和 HAE 登记的部分。遗传性血管性水肿会导致随机且通常不可预测的疼痛性肿胀发作，通常影响四肢、肠粘膜、生殖器、面部和上呼吸道。喉部发作与严重的功能障碍、健康相关的生活质量下降以及喉部发作的死亡率有关。由于 HAE 疾病的复杂性，护理该患者可能具有挑战性。与许多国家一样，加拿大对 HAE 患者的护理仍然既不理想也不统一。它落后于其他一些国家，这些国家的 HAE 管理模式更加有组织，并且更多获得许可的治疗方案可供选择。预计向护理人员、政策制定者、患者和倡导者提供本指南不仅将优化 HAE 的管理，而且还将提高个体化护理的重要性。 本指南的主要目标用户是管理 HAE 患者的医疗保健提供者。其他可能使用本指南的医疗保健提供者包括急诊和重症监护医生、初级保健医生、胃肠病学家、牙医、耳鼻喉科医生、儿科医生和妇科医生，他们会遇到 HAE 患者并需要了解这种情况。医院管理人员、保险公司和政策制定者也可能会发现该指南很有帮助。