目的 总结囊性纤维化（cystic fibrosis, CF）的胸部影像特征，加深对该病的认识，以期早期诊断和及时治疗。 方法 回顾性分析2012年7月至2016年11月在北京协和医院就诊的9例CF患者的临床及影像资料，分析其胸部影像征 象。结果 9例CF患者中病变均呈弥漫性，所有病例均累及双侧肺。9例病例中除1例患儿以双肺支气管管壁增厚为主 要肺部表现外，其余8例患者均以支气管扩张为肺部主要影像表现。支气管扩张以柱状支气管扩张为主，且以双肺 上叶病变较为严重。双肺周围部多发支气管管腔黏液嵌塞，可伴有马赛克样灌注、肺气肿、肺不张或肺实变等征 象。结论 肺囊性纤维化常被误诊，其胸部影像表现具有一定的特征性，对于反复出现肺部感染的年轻患者，应高 度警惕CF。

Objective To summarize the chest imaging features of cystic fibrosis, deepen the learning of the disease to achieve early diagnosis and timely treatment. Methods We retrospectively analyzed the clinical data and CT images of 9 patients who had been admitted to Peking Union Medical College Hospital from July 2012 to November 2016 due to CF. Results Among the 9 patients, the lesions were diffuse and all the cases affected bilaterally. The most common CT findings from the 9 patients were bronchiectasis except one little boy with bronchial wall thickening. In CF, bilateral columnar bronchiectasis with disease predominant in the upper lobe is routinely noted. The visibility of mucous plugging the bronchioles within 2cm of pleural surface, followed by mosaic perfusion, air trapping, emphysema, atelectasis and consolidation. Conclusion CF usually is misdiagnosed, and it shows some imaging characteristics. Clinicians should take CF into consideration when young patients had recurrent pulmonary infection.