气管支气管巨大症1例并文献复习  

Mounier-Kuhn syndrome or tracheobronchomegaly： a case report and literature review

作　　者：刘晶晶[1] 李园园[1] 黄瓅[1] 唐勇军[1] 邓彭博[1] 胡成平[1] 

机构地区：[1]中南大学湘雅医院呼吸内科,长沙410008

出　　处：《国际呼吸杂志》2015年第1期39-43,共5页International Journal of Respiration

摘　　要：目的 提高对气管支气管巨大症（tracheobronchomegaly,TBM）,即莫-昆综合征的认识,有利于早期诊断,及时治疗,改善预后。方法 对中南大学湘雅医院呼吸内科诊治的1例TBM 患者的临床资料进行回顾性分析,并结合相关文献报道加以总结。结果 ①TBM 是一种罕见病,病因及发病机制不明,多倾向于先天性起源。②TBM 以气管、主支气管的弹性纤维及平滑肌组织萎缩或发育不良为组织学改变,肺功能常表现为阻塞性通气功能障碍。③TBM 起病缓慢,主要表现为反复咳嗽、咳痰和进行性呼吸困难;因临床表现无特异性,误诊、漏诊率高。④TBM 的诊断主要依靠胸部高分辨率CT,表现为气管、主支气管显著扩张。⑤TBM 缺乏有效的治疗手段,主要是提高对该病的认识,预防和控制感染。结论 对于反复下呼吸道感染,临床表现类似于慢性支气管炎或支气管扩张的患者,应警惕TBM 的可能;提高对该病的认识,早期诊断并及时控制反复发作的肺部感染,对阻止肺功能下降、改善预后有重要帮助。Objective To improve the knowledge of tracheobronchomegaly （TBM, Mounier-Kuhn syndrome）. Methods One patient with TBM was analyzed, and the related literatures were reviewed. Results （1） TBM was an extremely rare disease, although the etiology was unknown, most scholars believed that TBM was a congenital disorder. （2）TBM was characterized by a congenital defect or atrophy of the elastic and smooth muscle tissue of the trachea and main bronchi. Pulmonary function tests showed obstructive ventilatory dysfunction. （3）The onset of TBM was slowly, and its most usual clinical manifestations included recurrent cough and sputum,and progressive dyspnea. Given the non-specific clinical pattern, the misdiagnosis rate of TBM is probably higher. （4）High-resolution CT scan of the chest was used for the diagnoses, which showed the trachea and the main bronchi marked dilatation. （5） Treatmeent was mainly supportive with chest physiotherapy and antibiotics. Conclusions TBM should be considered for patients who have recurrent pulmonary infection. A careful analysis of the chest radiograph of these patients is required. Raising awareness of the disease and diagnosis, it is important to help the disease diagnosis.

关 键 词：气管支气管巨大症 诊断 治疗 

分 类 号：R562[医药卫生—呼吸系统]