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遗传性血管性水肿喉发作误诊和管理不当的后果:罗马尼亚登记处病例概述

Consequences of Misdiagnosed and Mismanaged Hereditary Angioedema Laryngeal Attacks: An Overview of Cases from the Romanian Registry.

作者信息

Moldovan Dumitru, Bara Noémi, Nădășan Valentin, Gábos Gabriella, Mihály Enikő

机构信息

Romanian Network for Hereditary Angioedema, 11a Sântana St, 540256 Tîrgu-Mureş, Romania.

University of Medicine and Pharmacy, Mureş County Hospital, 1 Marinescu St, 540139 Tîrgu-Mureş, Romania.

出版信息

Case Rep Emerg Med. 2018 Oct 22;2018:6363787. doi: 10.1155/2018/6363787. eCollection 2018.

Abstract

Emergency department (ED) physicians frequently encounter patients presenting with angioedema. Most of these involve histamine-mediated angioedema; however, less common forms of angioedema (bradykinin-mediated) also occur. It is vital physicians correctly recognize and treat this; particularly since bradykinin-mediated angioedema does not respond to antihistamines, corticosteroids or epinephrine and hereditary angioedema (HAE) laryngeal attacks can be fatal. Here we present four case reports illustrating how failures in recognizing, managing, and treating laryngeal edema due to HAE led to asphyxiation and death of the patient. Recognition of the specific type of angioedema is critical for rapid and effective treatment of HAE attacks. Bradykinin-mediated angioedema should be efficiently differentiated from the most common histamine-mediated form. Improved awareness of HAE and the associated risk of life-threatening laryngeal edema among emergency physicians, patients, and relatives and clear ED treatment protocols are warranted. Moreover, appropriate treatments should be readily available to reduce fatalities associated with laryngeal edema.

摘要

急诊科(ED)医生经常会遇到出现血管性水肿的患者。其中大多数是由组胺介导的血管性水肿;然而,也会出现不太常见的血管性水肿形式(缓激肽介导)。医生正确识别并治疗这一病症至关重要;特别是因为缓激肽介导的血管性水肿对抗组胺药、皮质类固醇或肾上腺素无反应,而遗传性血管性水肿(HAE)的喉部发作可能是致命的。在此,我们呈现四个病例报告,说明由于未能识别、管理和治疗HAE导致的喉部水肿,如何致使患者窒息死亡。识别血管性水肿的特定类型对于快速有效地治疗HAE发作至关重要。缓激肽介导的血管性水肿应与最常见的组胺介导形式有效区分开来。急诊科医生、患者及其亲属应提高对HAE以及危及生命的喉部水肿相关风险的认识,并制定明确的急诊科治疗方案。此外,应随时提供适当的治疗方法,以减少与喉部水肿相关的死亡。

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