Geisel School of Medicine, Hanover, New Hampshire, USA.
Curr Opin Pediatr. 2012 Oct;24(5):638-46. doi: 10.1097/MOP.0b013e328357b25e.
To review and update the management and understanding of hereditary angioedema (HAE), while integrating insights into pediatric subtleties that exist in practice.
Major advances have recently been made in HAE treatment. Ecallantide (a kallikrein inhibitor approved for use in the United States in December 2009) and icatibant (a selective bradykinin B2 receptor antagonist approved for use in the United States in August 2011) represent novel subcutaneous therapies for acute HAE exacerbations. Recombinant human C1 esterase inhibitor (C1INH) serves as a promising future alternative to current mainstay acute and prophylactic treatment with plasma-derived C1INH. Recent guidelines have outlined new algorithms for short-term and long-term prophylaxis against HAE exacerbations.
The evolving standard of care for HAE management involves not only treatment of acute exacerbations but also individualized patient preference-sensitive short-term and long-term prophylaxis. Updated international consensus guidelines provide useful protocols, whereas recent clinical reviews have raised awareness of HAE. Further advances will likely focus on improving patient access to convenient acute and prophylactic treatment with C1INH.
在整合实践中儿科细微差别见解的同时，对遗传性血管性水肿（HAE）的管理和认识进行回顾和更新。
HAE 的治疗最近取得了重大进展。艾卡兰肽（一种激肽释放酶抑制剂，于 2009 年 12 月在美国获得批准使用）和依替巴肽（一种选择性缓激肽 B2 受体拮抗剂，于 2011 年 8 月在美国获得批准使用）是治疗急性 HAE 加重的新型皮下治疗药物。重组人 C1 酯酶抑制剂（C1INH）作为目前使用血浆衍生 C1INH 进行急性和预防性治疗的主要方法的替代方法，具有广阔的前景。最近的指南概述了针对 HAE 加重的短期和长期预防的新算法。
HAE 管理的不断发展的标准护理不仅包括急性加重的治疗，还包括个体化的患者偏好敏感的短期和长期预防。更新的国际共识指南提供了有用的方案，而最近的临床综述提高了对 HAE 的认识。进一步的进展可能集中在改善患者获得方便的急性和预防性 C1INH 治疗。
