Hereditary angioedema type III – case report

IF 0.2 Q4 ALLERGY

Alergologia Polska - Polish Journal of Allergology Pub Date : 2021-01-01 DOI:10.5114/PJA.2021.104656

Katarzyna Waligóra-Dziwak, M. Woźniak, D. Jenerowicz, Z. Adamski, M. Czarnecka-Operacz

引用次数: 0

Abstract

Hereditary angioedema (HAE) is a genetically determined disease characterized by recurrent episodes of subcu-taneous or submucosal tissue oedema. Failure to respond to the classical treatment of angioedema with antihista-mines, glucocorticosteroids as well as adrenaline is highly characteristic for HAE. Three main types of HAE have been described: type I – associated with hereditary reduction of C1 esterase inhibitor plasma concentration, type II – associated with its dysfunction and type III with both C1 inhibitor concentration and activity being within the normal range. We present a case report of a 16-year-old female patient with chronic spontaneous angioedema with normal activity and concentration of C1 inhibitor as well as an updated review of the current knowledge concerning pathophysiology, clinical genetic HAE type

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遗传性血管性水肿III型1例报告

遗传性血管性水肿(HAE)是一种由遗传决定的疾病，其特征是皮下或粘膜下组织水肿反复发作。抗组胺药、糖皮质激素和肾上腺素对血管水肿的经典治疗无效是HAE的高度特征。已经描述了三种主要类型的HAE: I型与遗传性C1酯酶抑制剂血浆浓度降低有关，II型与C1酯酶抑制剂功能障碍有关，III型C1酯酶抑制剂浓度和活性均在正常范围内。我们报告了一例16岁的慢性自发性血管性水肿女性患者，其C1抑制剂活性和浓度正常，并对目前有关病理生理学、临床遗传HAE类型的知识进行了最新综述

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Alergologia Polska - Polish Journal of Allergology ALLERGY-

自引率

50.00%

发文量

期刊介绍： Alergologia Polska - Polish Journal of Allergology is aimed mainly at allergologists, but also medical doctors working in related fields, such as otolaryngology, pulmonology, and dermatology. The main goal of the journal is to ensure rapid publication of important research papers and interesting case studies from the following areas: allergology, diagnostics, therapy of allergic diseases, in particular in the area of immunotherapy, rhinitis, asthma. The Editorial Board accepts for publication original papers, case studies and letters to the Editor. We also publish review articles (both commissioned and those agreed upon with the Editor-in-Chief), articles dealing with standards of medical practice, as well as special issues. The journal is published quarterly. We guarantee short review times (up to two weeks) and immediate publication on-line upon Editor acceptance.