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自发性纵隔气肿的一种罕见病因:穆尼耶-库恩综合征。

An Unusual Cause of Spontaneous Pneumomediastinum: The Mounier-Kuhn Syndrome.

作者信息

Naciri Salim, Zahraoui Rachida, Soualhi Mouna, Bourkadi Jamal-Eddine

机构信息

Moulay Youssef Hospital, Pulmonary Unit, University Mohammed V of Rabat, Morocco.

出版信息

Case Rep Pulmonol. 2019 Jul 8;2019:5359309. doi: 10.1155/2019/5359309. eCollection 2019.

Abstract

Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. An 81-year-old man presenting with an acute chest pain was referred to the pulmonology department. His chest computed tomographic scan showed a tracheobronchomegaly with an increase in the diameter of both the trachea and right and left main bronchi, associated with pneumomediastinum and fibrosis. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchi. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Besides considering this long-neglected "orphan disease" when diagnosing spontaneous pneumomediastinum, clinicians should also be aware of an underlying Mounier-Kuhn syndrome in patients with recurrent respiratory infections, in order to avoid complications associated with the disease.

摘要

穆尼耶 - 库恩综合征是一种罕见的临床和影像学病症。其特征为气管和支气管扩张。诊断通过计算机断层扫描和支气管镜检查进行。一名81岁因急性胸痛就诊的男性被转诊至呼吸内科。他的胸部计算机断层扫描显示气管支气管巨大症,气管及左右主支气管直径均增大,伴有纵隔气肿和纤维化。纤维支气管镜检查显示气管和双侧主支气管均扩大。这些发现符合穆尼耶 - 库恩综合征的诊断。在诊断自发性纵隔气肿时,临床医生除了应考虑这种长期被忽视的“罕见病”外,对于反复发生呼吸道感染的患者,还应意识到其潜在的穆尼耶 - 库恩综合征,以避免该疾病相关的并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7120/6644234/eca81bf64f26/CRIPU2019-5359309.001.jpg

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