最新的叙述回顾:穆尼埃-库恩综合征和气管支气管肥大。

IF 3.5 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Shivang Sharma, Stephen J Kuperberg
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引用次数: 0

摘要

穆尼尔-库恩综合征(MKS)或气管支气管肿大是一种罕见的中央气道疾病。它的特点是气管和主支气管的病理性扩张,不可避免地导致复发性呼吸道感染、支气管扩张、住院治疗,并导致相当高的发病率和死亡率。尽管有大量病例报告,但临床结果证据不足,干预措施数据有限,因此在文献中存在重大空白。幸运的是,新的策略和不断增加的临床经验已经改善了MKS的临床方法、诊断检查、分类和管理。从Pubmed、谷歌Scholar、Medline和SCOPUS等数据库中检索英语、西班牙语和法语的文章,检索词为“Mounier-Kuhn综合征”、“气管肿大”、“气管支气管肿大”和“支气管肿大”,没有日期限制。Pubmed上总共索引了360篇具有上述语法的文章。这篇最新的综述试图通过总结当前的科学知识和强调新的干预策略来填补当前文献中的空白。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
State-of-the-art narrative review: Mounier-Kuhn syndrome and tracheobronchomegaly.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly is an uncommon disease of the central airways. It is characterized by pathological dilatation of the trachea and main bronchi and inevitably leads to recurrent respiratory infections, bronchiectasis, hospitalizations, and results in considerable morbidity and mortality. Despite numerous case reports, there is a shortage of evidence on clinical outcomes and limited data on interventions, thus presenting a significant gap in the literature. Fortunately, new strategies and increasing clinical experience have improved the clinical approach, diagnostic workup, classification, and management of MKS. Articles in English, Spanish, and French were searched from databases, including Pubmed, Google Scholar, Medline, and SCOPUS, using the terms "Mounier-Kuhn syndrome," "Tracheomegaly," "Tracheobronchomegaly," and "Bronchomegaly," without date restrictions. A total of 360 articles with the aforementioned syntax were indexed on Pubmed. This state-of-the-art review attempts to fill a void in the current literature by summarizing the current scientific knowledge and highlighting novel interventional strategies in the management of Mounier Kuhn Syndrome.

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来源期刊
Respiratory medicine
Respiratory medicine 医学-呼吸系统
CiteScore
7.50
自引率
0.00%
发文量
199
审稿时长
38 days
期刊介绍: Respiratory Medicine is an internationally-renowned journal devoted to the rapid publication of clinically-relevant respiratory medicine research. It combines cutting-edge original research with state-of-the-art reviews dealing with all aspects of respiratory diseases and therapeutic interventions. Topics include adult and paediatric medicine, epidemiology, immunology and cell biology, physiology, occupational disorders, and the role of allergens and pollutants. Respiratory Medicine is increasingly the journal of choice for publication of phased trial work, commenting on effectiveness, dosage and methods of action.
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