Johnson Nathan M, Phillips Mariana A
Virginia Tech Carilion School of Medicine, Roanoke, VA, USA; Carilion Clinic Dermatology & Mohs Surgery, Roanoke, VA, USA.
Skin Therapy Lett. 2018 Jan;23(1):6-8.
Hereditary angioedema is characterized by severe, episodic edema of the subcutaneous and mucosal tissue. The disease carries significant morbidity and mortality due to involvement of the gastrointestinal tract and upper airway. Recent advances in the treatment of hereditary angioedema include new techniques used to isolate and purify human-derived C1 inhibitor, the production of a recombinant form of C1 inhibitor, and the development of drugs that target the kallikrein-kinin pathway. This paper reviews the mechanisms, efficacy, and adverse reactions associated with these medications.
遗传性血管性水肿的特征是皮下和黏膜组织出现严重的发作性水肿。由于胃肠道和上呼吸道受累，该疾病具有显著的发病率和死亡率。遗传性血管性水肿治疗的最新进展包括用于分离和纯化人源C1抑制剂的新技术、重组形式C1抑制剂的生产以及针对激肽释放酶-激肽途径的药物开发。本文综述了与这些药物相关的作用机制、疗效和不良反应。
