通讯作者: 罗胜, tcm2007@163.com

DOI：10.12201/bmr.202504.00057

Mounier-Kuhn syndrome: report of two cases and literature review

• 摘要：【】目的:分析总结Mounier-Kuhn综合征(MKS)的病因及临床特点,以提高对该疾病的认知。方法:报道本院确诊的两例MKS患者,分析其临床特点及影像学表现,并检索CNKI、万方、Pubmed、Web of Science数据库,筛选出高质量MKS病例报告进行系统评价。结果:共纳入240例MKS患者,其病因尚未明确,男性好发,病理特征为气管、主支气管的弹性纤维和平滑肌组织萎缩、纤细,胸部CT、气管镜可见明显的气管支气管扩张,临床表现无特异性。该病缺乏有效治疗手段,目前以抗感染治疗为主,少数可行气管支架置入。结论:MKS是一种罕见呼吸道疾病,与吸烟史无关,可通过胸部CT或气管镜诊断,对于无明显诱因的反复下呼吸道感染应注意本病可能。

  关键词： Mounier-Kuhn综合征; 文献复习

   

  Abstract: 【 】 Objective: To analyze and summarize the etiology and clinical characteristics of Mounier-Kuhn syndrome (MKS), so as to improve the understanding of the disease. Methods: Two cases of MKS diagnosed in our hospital were reported, and their clinical features and imaging findings were analyzed. CNKI, Wanfang, Pubmed, and Web of Science databases were searched to screen out high-quality case reports of MKS for systematic review. Results: A total of 240 patients with MKS were included in this study. The etiology of MKS was not clear, and males were more common. The pathological features were atrophy and slender elastic fibers and smooth muscle tissue of the trachea and main bronchus, and obvious tracheal bronchiectasis was seen on chest CT and bronchoscopy. There is no effective treatment for this disease. At present, anti-infective therapy is the main treatment, and tracheal stent implantation is feasible in a few cases. Conclusions: MKS is a rare respiratory disease, which is not related to smoking history. It can be diagnosed by chest CT or bronchoscopy.

  Key words: Mounier-Kuhn syndrome; Literature review

  提交时间：2025-04-18

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• 序号	提交日期	编号	操作
  1	2025-02-20	10.12201/bmr.202504.00057V1	下载

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