Kuwal Ashok, Dutt Naveen, Chauhan Nishant, Kumar Sunil, Purohit Swetabh, Saini Lokesh K
Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.
J Bronchology Interv Pulmonol. 2017 Jan;24(1):84-87. doi: 10.1097/LBR.0000000000000226.
Mounier-Kuhn syndrome (MKS) is a rare disease of unknown etiology characterized by abnormal pathologic dilatation of tracheabronchial tree. The diagnosis of MKS is normally made on CT scan of chest on the basis of enlarged diameters of trachea and main stem bronchi. We are presenting histologically confirmed case of MKS, where the diameter of right main bronchus is below minimum diameter (mean+3 SD) required for the diagnosis. We suggest that the diagnosis of MKS should not be solely based on fixed criteria such as the diameter of airways, but on the basis of the overall clinical, pathologic, and radiologic profile.
穆尼耶-库恩综合征（MKS）是一种病因不明的罕见疾病，其特征为气管支气管树的病理性异常扩张。MKS的诊断通常基于胸部CT扫描中气管和主支气管直径增大的情况。我们现报告一例经组织学确诊的MKS病例，该病例中右主支气管直径低于诊断所需的最小直径（均值+3标准差）。我们建议，MKS的诊断不应仅基于气道直径等固定标准，而应基于整体的临床、病理和放射学特征。
