icatibant成功治疗产后遗传性血管性水肿

IF 1.1 Q4 ALLERGY

Journal of Cutaneous Immunology and Allergy Pub Date : 2022-07-07 DOI:10.1002/cia2.12267

Yukari Kondo MD, Aki Yoshimura MD, Noriko Azuma MD

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引用次数: 0

摘要

遗传性血管性水肿(HAE)是一种临床症状多变的罕见疾病。女性患者往往受到更严重的影响，因为雌激素在血管性水肿发作中起作用。尽管在围产期处理HAE的紧迫性，但由于缺乏基于病例的报告，使用icatibant(一种缓激肽受体拮抗剂)的安全性尚未得到证实一名日本女性因反复发作的呼吸困难、腹痛、手指和面部水肿而被转介至我院(图1A)。她根据家族史被诊断为HAE, C1INH功能活性下降(<25%;正常，70% - 130%)和C4水平(5.0 mg/ dl;正常，1134 mg/dl)，计算机断层扫描显示肠壁厚度增加(图1B)。她在急症室接受了偶尔的按需静脉注射1000 IU等离子体合成C1INH (pdC1INH)的治疗。她在35岁时通过人工受精怀孕。在怀孕期间，她通过按需注射pdC1INH来控制HAE发作。在分娩和其他侵入性医疗过程中，短期预防使用pdC1INH，这是当时日本唯一接受的妊娠HAE患者的治疗方法。36岁时，她生了孩子并开始母乳喂养。分娩后4个月，在知情同意并被告知icatibant在母乳喂养期间的风险和益处后，她开始皮下自我施用icatibant 30mg，作为产后HAE发作的管理。Icatibant

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Successful management of hereditary angioedema with icatibant during the postpartum period

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Successful management of hereditary angioedema with icatibant during the postpartum period

A. Facial swelling during a hereditary angioedema (HAE) attack. B. Computed tomography image of the abdomen with intestinal edema during an HAE attack. C. Histogram of the frequency of monthly HAE attacks during self-administration of icatibant. The asterisk indicates when HAE attacks were treated by on-demand injection of plasma-derived C1 inhibitor at the emergency room.

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来源期刊

Journal of Cutaneous Immunology and Allergy Medicine-Dermatology

CiteScore

0.60

自引率

10.00%

发文量

审稿时长

12 weeks