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声门下及气管上段的韦格纳肉芽肿病

Wegener's granulomatosis of the subglottis and the upper portion of the trachea.

作者信息

McDonald T J, Neel H B, DeRemee R A

出版信息

Ann Otol Rhinol Laryngol. 1982 Nov-Dec;91(6 Pt 1):588-92. doi: 10.1177/000348948209100610.

Abstract

A relatively little-known complication of Wegener's granulomatosis is involvement of the subglottis and upper part of the trachea, which is illustrated in this report on 17 patients. Each of the patients had biopsy-proved Wegener's granulomatosis of the nose and paranasal sinuses. Some had concomitant involvement of the eye, kidney, lung and skin. Nine of the 17 patients required tracheotomy; 5 still require a tracheotomy tube, and 4 have had decannulation (1 had successful reconstructive surgery of the trachea). Medical treatment consisted of cyclophosphamide, with or without corticosteroids. All 17 are currently alive, the average follow-up having been 81 months. The otolaryngologist plays a key role in the detection and management of patients with Wegener's granulomatosis, particularly when the onset is a sudden progressive upper tracheal obstruction with airway symptoms that may overshadow the sometimes more subtle nasal manifestations.

摘要

韦格纳肉芽肿一种相对鲜为人知的并发症是声门下和气管上部受累,本报告对17例患者进行了阐述。每例患者均经活检证实患有鼻和鼻窦韦格纳肉芽肿。部分患者同时累及眼、肾、肺和皮肤。17例患者中有9例需要气管切开术;5例仍需留置气管切开套管,4例已拔管(1例成功进行了气管重建手术)。药物治疗包括环磷酰胺,可联合或不联合使用皮质类固醇。17例患者目前均存活,平均随访时间为81个月。耳鼻喉科医生在韦格纳肉芽肿患者的检测和管理中起着关键作用,尤其是当起病为突然进行性的上气管梗阻并伴有气道症状,而这些症状可能掩盖有时更为隐匿的鼻部表现时。

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