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2型遗传性血管性水肿表现为急性鼻-鼻窦炎的眼眶并发症。

Hereditary angioedema type 2 presented as an orbital complication of acute rhinosinusitis.

作者信息

Somuk Battal Tahsin, Göktas Göksel, Özer Samet, Sapmaz Emrah, Bas Yalcın

机构信息

Department of Otorhinolaryngology, Gaziosmanpasa University School of Medicine, Tokat, Turkey.

Department of Otorhinolaryngology, Gaziosmanpasa University School of Medicine, Tokat, Turkey.

出版信息

Int J Pediatr Otorhinolaryngol. 2016 Mar;82:16-8. doi: 10.1016/j.ijporl.2015.12.030. Epub 2016 Jan 8.

Abstract

Hereditary angioedema is an autosomal dominant and life-threatening disorder characterized by recurrent episodes of non-pitting edema affecting the skin, respiratory system and digestive tracts and caused by a congenital deficiency or function defect of the C1 esterase inhibitor. Preseptal cellulitis is defined as an infection of the tissues of the anterior orbital septum. It is generally caused by complications from an upper respiratory tract infection, dacryocystitis, dermal infection, and, rarely, sinusitis. The disease presents with orbital pain, edema on the eyelids, erythema, and fever. In this case, a child with hereditary angioedema type 2 who presented as mimicking a complication of acute sinusitis is discussed.

摘要

遗传性血管性水肿是一种常染色体显性遗传且危及生命的疾病,其特征为非凹陷性水肿反复发作,累及皮肤、呼吸系统和消化道,由C1酯酶抑制剂先天性缺乏或功能缺陷所致。眶隔前蜂窝织炎被定义为眶前间隔组织的感染。它通常由上呼吸道感染、泪囊炎、皮肤感染以及罕见的鼻窦炎并发症引起。该病表现为眼眶疼痛、眼睑水肿、红斑和发热。本文讨论了一名表现为类似急性鼻窦炎并发症的2型遗传性血管性水肿患儿。

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