Case Report: A case of reversible tracheal diameter Mounier-Kuhn syndrome and literature review.

IF 3.1 3区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Frontiers in Medicine Pub Date : 2025-04-28 eCollection Date: 2025-01-01 DOI:10.3389/fmed.2025.1544843
Lu-Xia Kong, Zhen-Hua Li, Ji-Xiang Ni
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引用次数: 0

Abstract

Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly (TBM) or tracheomegaly, is an extremely rare and chronic airway disease characterized by significant dilation of the trachea and central bronchi. Currently, there is a paucity of epidemiological studies on MKS, with the majority of data derived from case reports, resulting in limited understanding of this disease among clinicians. We encountered an 81-year-old male MKS patient whose diagnosis was delayed, and subsequently experienced a significant decrease in tracheal diameter post-diagnosis, marking the first documented case of reversible tracheal diameter in MKS patients. Concurrently, we conducted a review of 76 cases published within the past decade to procure comprehensive insights into the clinical characteristics of MKS patients. Based on our findings, we draw two key conclusions. First, at present, clinicians and radiologists have insufficient understanding of this disease, which often leads to missed diagnosis, misdiagnosis or delayed diagnosis. Second, the tracheal diameter of MKS has the potential to be reversible with appropriate treatment. This is the first case where the tracheal diameter has been found to be reversible.

病例报告:可逆性气管直径Mounier-Kuhn综合征1例并文献复习。
Mounier-Kuhn综合征(MKS),也被称为气管支气管肿大(TBM)或气管肿大,是一种极其罕见的慢性气道疾病,其特征是气管和中央支气管明显扩张。目前,关于MKS的流行病学研究缺乏,大多数数据来自病例报告,导致临床医生对这种疾病的了解有限。我们遇到了一位81岁的男性MKS患者,他的诊断被延迟,随后在诊断后气管直径明显减少,这是MKS患者中第一例气管直径可逆的病例。同时,我们对过去十年发表的76例病例进行了回顾,以全面了解MKS患者的临床特征。基于我们的发现,我们得出了两个关键结论。首先,目前临床医生和放射科医师对本病认识不足,常常导致漏诊、误诊或延误诊断。其次,通过适当的治疗,MKS的气管直径有可能是可逆的。这是第一例气管直径可逆的病例。
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来源期刊
Frontiers in Medicine
Frontiers in Medicine Medicine-General Medicine
CiteScore
5.10
自引率
5.10%
发文量
3710
审稿时长
12 weeks
期刊介绍: Frontiers in Medicine publishes rigorously peer-reviewed research linking basic research to clinical practice and patient care, as well as translating scientific advances into new therapies and diagnostic tools. Led by an outstanding Editorial Board of international experts, this multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. In addition to papers that provide a link between basic research and clinical practice, a particular emphasis is given to studies that are directly relevant to patient care. In this spirit, the journal publishes the latest research results and medical knowledge that facilitate the translation of scientific advances into new therapies or diagnostic tools. The full listing of the Specialty Sections represented by Frontiers in Medicine is as listed below. As well as the established medical disciplines, Frontiers in Medicine is launching new sections that together will facilitate - the use of patient-reported outcomes under real world conditions - the exploitation of big data and the use of novel information and communication tools in the assessment of new medicines - the scientific bases for guidelines and decisions from regulatory authorities - access to medicinal products and medical devices worldwide - addressing the grand health challenges around the world
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