Centre of Pulmonary Diseases, Pauls Stradins Clinical University Hospital, Pilsonu Street 13, Riga LV1002, Latvia.
Respir Med. 2013 Dec;107(12):1822-8. doi: 10.1016/j.rmed.2013.08.042. Epub 2013 Sep 6.
Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males. It is commonly overlooked on conventional chest X-rays, and is considered to be rare, but the prevalence might be higher as commonly assumed. The hallmark of it is a dilatation of the main airways which frequently, but not always, causes marked, mainly respiratory, symptoms, and patients usually present with varying degrees of recurrent infections, breathlessness, haemoptysis, dyspnoea. Although at least 200 case reports have been published, there have been only a few attempts to review them, and none in the last 20 years. Due to the lack of clinical trials and wide variability of case-report format, a systematic review was deemed not feasible, therefore PubMed and Medline databases were searched using terms "Mounier-Kuhn syndrome", "tracheobronchomegaly", "tracheomegaly", and "bronchomegaly", without any time restrictions, to summarize currently known facts about the syndrome. To the authors' best knowledge, the result is currently the most comprehensive review of previously published literature about the congenital tracheobronchomegaly, and summarizes what's known about symptoms, prevalence, disease associations, and treatment options for this syndrome.
Mounier-Kuhn 综合征或先天性气管支气管巨大症是一种慢性气道疾病，其病因目前尚不清楚，但主要影响男性。在常规胸部 X 光片上通常容易被忽视，并且被认为较为罕见，但实际的患病率可能比普遍认为的要高。其主要特征是主要气道扩张，这常常导致明显的、主要是呼吸方面的症状，患者通常表现为不同程度的反复感染、呼吸困难、咯血、呼吸困难。尽管已经发表了至少 200 份病例报告，但只有少数几次尝试对其进行综述，而且最近 20 年来都没有。由于缺乏临床试验和病例报告格式的广泛变异性，因此认为进行系统综述是不可行的，因此使用“Mounier-Kuhn 综合征”、“气管支气管巨大症”、“气管扩张”和“支气管扩张”等术语在 PubMed 和 Medline 数据库中进行了无时间限制的搜索，以总结目前关于该综合征的已知事实。据作者所知，这是目前对先天性气管支气管巨大症已发表文献的最全面综述，总结了该综合征的症状、患病率、疾病相关性和治疗选择方面的已知信息。
