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中华肺部疾病杂志(电子版) ›› 2023, Vol. 16 ›› Issue (05) : 748 -751. doi: 10.3877/cma.j.issn.1674-6902.2023.05.040

病例报告

以体位相关性咳喘为主要症状的女性Mounier-Kuhn综合征一例并文献复习
翁晓芹, 童兰庭, 张扬, 秦涌()   
  1. 635016 江苏,南京医科大学第二附属医院
  • 收稿日期:2023-04-10 出版日期:2023-10-25
  • 通信作者: 秦涌

One case of Mounier-Kuhn syndrome in female with postulation-related cough and literature review

Xiaoqin Weng, Lanting Tong, Yang Zhang   

  • Received:2023-04-10 Published:2023-10-25
引用本文:

翁晓芹, 童兰庭, 张扬, 秦涌. 以体位相关性咳喘为主要症状的女性Mounier-Kuhn综合征一例并文献复习[J]. 中华肺部疾病杂志(电子版), 2023, 16(05): 748-751.

Xiaoqin Weng, Lanting Tong, Yang Zhang. One case of Mounier-Kuhn syndrome in female with postulation-related cough and literature review[J]. Chinese Journal of Lung Diseases(Electronic Edition), 2023, 16(05): 748-751.

Mounier-Kuhn综合征(MKS)又称为气管支气管巨大症(tracheobronchomegaly, TBM),是一种罕见的呼吸系统疾病,以不明原因的气管、支气管扩大为主要特征,通常伴有气管、支气管憩室、呼气相气道塌陷、支气管扩张及反复的下呼吸道感染[1]。其临床症状不典型,发病率低,在临床中极容易漏诊及误诊,本文报道1例并通过复习相关文献对MKS的病因学、病理生理机制、临床表现、诊治情况及相关知识进行总结。

图1 吸气相、呼气相CT,吸气时气管及左右主支气管扩张明显,呼气相见气道明显塌陷,气管及左右支气管塌陷最为明显。注:A:吸气相;B:呼气相
图2 气管镜检查。注:A:气管(呼气相);B:气管(呼气相);C:隆突、左右主支气管(吸气相);D:隆突、左右主支气管(呼气相);E:右上叶支气管(前支见痰栓);F:右中间段支气管(见痰栓阻塞);G:左上叶支气管(左舌叶狭窄);H:左下叶支气管(基底段见大量痰栓)
图3 "黏膜活检"支气管黏膜慢性炎
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