这是对2014年加拿大遗传性血管性水肿指南的更新,范围扩大,包括全世界遗传性血管性水肿(HAE)患者的管理。这是加拿大和国际HAE专家和由加拿大遗传性血管性水肿网络领导的患者团体的合作。本指南的目的是提供基于证据的建议,使用等级制度,用于HAE患者的管理。这包括治疗攻击,短期预防,长期预防,以及自我管理的建议,个体化治疗,生活质量,全面的护理。本指南2019年版的新内容是涵盖C1-INH正常的HAE患者急性治疗的诊断和推荐治疗的部分。以及孕妇和儿科患者的章节,患者协会和HAE注册表。遗传性血管性水肿导致随机且通常不可预测的疼痛性肿胀发作,通常会影响四肢。肠粘膜,生殖器,面部和上呼吸道。攻击与严重的功能障碍有关,与健康相关的生活质量下降,和死亡率在喉部发作的情况下。由于这种疾病的复杂性,照顾患有HAE的患者可能是具有挑战性的。加拿大HAE患者的护理,和许多国家一样,继续既不是最优的,也不是统一的。它落后于其他一些国家,那里有更多有组织的HAE管理模式,以及更多获得许可的其他治疗选择。预计向护理人员提供这一指南,政策制定者,病人,倡导者不仅会优化HAE的管理,同时也促进了个性化护理的重要性。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是急诊和重症监护医师,初级保健医生,胃肠病学家,牙医,耳鼻喉科医师,儿科医生,和妇科医生将遇到HAE患者,需要了解这种情况。医院管理员,保险公司和政策制定者也可能会发现这一指导方针很有帮助。
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.