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耳部症状作为韦格纳肉芽肿病的首发表现:诊断困境。

Otologic symptoms as initial manifestation of wegener granulomatosis: diagnostic dilemma.

机构信息

ENT, Head Neck Oncological Surgery, Poznan University of Medical Sciences, Przybyszewskiego Street 49, 60-355 Poznan, Poland.

出版信息

Otol Neurotol. 2011 Aug;32(6):996-1000. doi: 10.1097/MAO.0b013e31822558fd.

Abstract

OBJECTIVE

To show 7 cases of Wegener granulomatosis (WG) with early aural symptoms and to discuss the problems of otologic manifestation in WG.

STUDY DESIGN

Retrospective case review.

SETTING

: Tertiary care university hospital.

PATIENTS

All patients were administered to the ENT University Department in Poznań in years 2002-2008 because of otitis media with effusion, facial palsy, sensorineural profound hypoacusis, hypoacusis combined with purulent discharge, and facial nerve palsy or progression of mixed type hypoacusis.

INTERVENTIONS

Diagnostics and treatment. MAIN OUTCOME MEASURESL: Otologic symptoms as initial manifestation of WG diagnostic dilemma.

RESULTS

The authors want to underline the young age of the patients, ranging from 32 to 46 years. The outcome of initially otologic cases, which developed generalized form of WG, was poor (the first patient died after 2 months, the second patient died after 7 days, the third had the pulmonary insufficiency in 2 months of observation, and the fourth had severe renal failure in 1 month), whereas the patients with localized disease have been successfully under control for 1 to 5 years.

CONCLUSION

As WG often presents otologic symptoms, as an initial sign in some cases, it is important to take WG into consideration in atypical inflammatory states of the ear. The otologic onset of WG is very insidious, and prompt diagnosis in early stage of disease is a challenge. Focal and localized disease in the aural region might possibly require less aggressive therapy than acute-onset multi-organ disease and is connected with better prognosis.

摘要

目的

展示 7 例以耳部早期症状为表现的韦格纳肉芽肿(WG),并讨论 WG 中耳科表现的问题。

研究设计

回顾性病例分析。

设置

三级护理大学医院。

患者

所有患者均因分泌性中耳炎、面瘫、感音神经性重度听力损失、伴有脓性分泌物的听力损失、面神经瘫痪或混合性听力损失进展,于 2002-2008 年在波兹南 ENT 大学系接受治疗。

干预措施

诊断和治疗。

主要观察指标

耳部症状作为 WG 诊断困境的首发表现。

结果

作者想强调患者的年龄较轻,为 32-46 岁。最初表现为耳部疾病且发展为全身性 WG 的患者的结局较差(第一例患者在 2 个月后死亡,第二例患者在 7 天后死亡,第三例患者在 2 个月的观察期内发生了肺功能不全,第四例患者在 1 个月内发生了严重的肾衰竭),而局限于耳部疾病的患者已成功控制病情 1-5 年。

结论

由于 WG 常表现为耳部症状,且在某些情况下为首发症状,因此在耳部不典型炎症状态下,应考虑 WG 的可能性。WG 的耳部发病非常隐匿,早期诊断具有挑战性。耳部局限性和局限性疾病可能需要的侵袭性治疗比急性发作的多器官疾病少,并且与更好的预后相关。

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