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Icatibant 自我给药治疗 I 型遗传性血管性水肿急性发作:一例报告并复习遗传性血管性水肿。

Self-administration of icatibant in acute attacks of Type I hereditary angioedema: A case report and review of hereditary angioedema.

机构信息

Department of Medical Sciences and Public Health, Unit of Dermatology, University of Cagliari, Cagliari, Italy.

Department of Dermatology, University Hospital of Coimbra, Coimbra, Portugal.

出版信息

Dermatol Ther. 2019 Nov;32(6):e13098. doi: 10.1111/dth.13098. Epub 2019 Oct 28.

Abstract

Hereditary angioedema (HAE) is a rare group of genetic disease characterized by non-itchy swelling of subcutaneous and submucosal tissues of the extremities, genitalia, gastrointestinal tract, and upper airways, which can be life threatening. Moreover, unpredictability and recurrence of HAE attacks significantly affect patients' quality of life. Short- and long-term prophylaxis is used to decrease the severity and frequency of attacks, but during severe or potentially severe acute episodes, treatment with C1-INH replacement or icatibant is mandatory. Icatibant is a selective bradykinin B2 receptor antagonist that has been licensed for self-administration at home, resulting in earlier treatment of the attack and quicker recovery, less emergency admittance with a significant improvement of patients' quality of life, and decrease of health care costs. The authors present a case of a young woman, affected by Type I HAE, who has been successfully treated with icatibant on demand at home, resulting in reduction of emergency admissions and improvement of quality of life. The authors also review the different types HAE, their clinical aspects, diagnosis, and management.

摘要

遗传性血管性水肿(HAE)是一组罕见的遗传性疾病,其特征为四肢、生殖器、胃肠道和上呼吸道的皮下和粘膜下组织无瘙痒性肿胀,可能危及生命。此外,HAE 发作的不可预测性和复发性严重影响患者的生活质量。短期和长期预防措施可降低发作的严重程度和频率,但在严重或潜在严重的急性发作期间,必须使用 C1-INH 替代物或艾替班特进行治疗。艾替班特是一种选择性缓激肽 B2 受体拮抗剂,已获准在家中自行给药,从而更早地开始治疗发作,更快地恢复,减少急诊入院,显著改善患者的生活质量,并降低医疗保健费用。作者报告了一例年轻女性,患有 I 型 HAE,在家中按需使用艾替班特成功治疗,减少了急诊入院次数,改善了生活质量。作者还回顾了不同类型的 HAE、其临床方面、诊断和管理。

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