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2020 年缓激肽介导的血管性水肿更新。

Update on bradykinin-mediated angioedema in 2020.

机构信息

Centre régional de pharmacovigilance, centre hospitalier universitaire Grenoble-Alpes, 38000 Grenoble, France.

Centre régional de pharmacovigilance, centre hospitalier universitaire Grenoble-Alpes, 38000 Grenoble, France.

出版信息

Therapie. 2020 Apr;75(2):195-205. doi: 10.1016/j.therap.2020.02.011. Epub 2020 Feb 13.

Abstract

Bradykinin-mediated angioedema is a rare disease, due to vasodilation and increased vascular permeability resulting from bradykinin. This kind of angioedema affects abdominal and/or upper airways. It differs clinically from histamine-mediated angioedema by the absence of urticaria or skin rash. Antihistamines and corticosteroids are not effective. Delayed diagnosis can lead to inadequate and potentially fatal management by asphyxiation. Bradykinin-mediated angioedema results from either overproduction of bradykinin or inhibition of its degradation. Etiology can be hereditary or acquired. Deficiency of C1 inhibitor and drug induced are the main causes of bradykinin-mediated angioedema. Its diagnosis is clinical (presentation, family history, seriousness, frequency, etc.) and biological (dosage of C1-INH level, C1-INH activity, and complement protein 4 level). Acute attack treatment is based on C1-inhibitor concentrates and icatibant, a bradykinin receptor antagonist. Long-term prophylaxis can be necessary, especially before surgical and dental procedures. New drugs, including gene therapy, are being tested.

摘要

缓激肽介导的血管性水肿是一种罕见的疾病,由于缓激肽导致血管扩张和血管通透性增加。这种血管性水肿影响腹部和/或上呼吸道。它与由组胺介导的血管性水肿在临床上不同,因为它没有荨麻疹或皮疹。抗组胺药和皮质类固醇无效。延迟诊断可能导致窒息导致的管理不足和潜在的致命性。缓激肽介导的血管性水肿是由于缓激肽的过度产生或其降解的抑制所致。病因可以是遗传性的或获得性的。C1 抑制剂缺乏和药物诱导是缓激肽介导的血管性水肿的主要原因。其诊断是临床的(表现、家族史、严重程度、频率等)和生物学的(C1-INH 水平、C1-INH 活性和补体蛋白 4 水平的剂量)。急性发作的治疗基于 C1 抑制剂浓缩物和依卡替班,一种缓激肽受体拮抗剂。长期预防可能是必要的,特别是在手术和牙科手术之前。正在测试新的药物,包括基因治疗。

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