Kartagener's综合征伴细支气管炎:影像诊断及随访

Bronchiolitis in Kartagener syndrome:imaging diagnosis and following up

目的探讨Kartagener综合征(KS)与细支气管炎的关系。方法回顾性分析24例KS的临床特点、胸部和鼻旁窦的影像学及病理表现。结果①24例KS中,19例均有支气管扩张、全内脏转位、鼻旁窦炎三联征,1例有支气管扩张和鼻旁窦炎,4例有全内脏转位和支气管扩张。临床症状均为反复咳嗽、咳痰、活动后气促,肺部均可闻及湿罗音,22例肺过度通气。②22例胸部DR、18例CT显示两肺弥漫分布2～5mm的微小结节影,胸部HRCT可见线样征和树芽征,14例(58.33%)表现类似弥漫性泛细支气管炎;20例(83.33%)有鼻旁窦炎。③8例病理确诊为弥漫性泛细支气管炎,病理特点为终末细支气管和呼吸性细支气管管壁增厚,慢性炎细胞浸润;其中2例透射电镜下可见纤毛的内侧臂缺失,其中1例伴有纤毛畸形、融合成团。结论KS主要表现为支气管扩张、内脏转位、鼻旁窦炎三联征,细支气管炎应作为KS的特征性表现之一,KS也可伴发弥漫性泛细支气管炎。

Objective To observe the association of diffuse bronchiolits and Kartagener syndrome（KS）.Methods Clinical manifestation,imaging examination and pathology results of 24 patients of KS were retrospectively reviewed.Results ①Nineteen patients of KS had classical triad of bronchiectasis,pansinusits and situs inversus,1 had bronchiectasis and pansinus,4 had bronchiectasis and situs inverus.All the patients had persistent cough,sputum production and exertion dyspnea.Coarse crackles were audible upon auscultation of the chest in all patients,while hyperinflation appeared in 22 patients.② DR of 22 patients and chest CT of 18 patients showed centrilobular small nodules with 2-5 mm in diameter in both lungs,and both tramlines and tree-bud sign were found on HRCT.HRCT dected lesions like diffuse panbronchiolitis（DPB）in 14,and chronic sinusitis in 20（83.33%）patients.③Eight patients were diagnosed as DPB with pathology,and thickening of the walls of the terminal bronchioles and respiratory bronchioles with infiltration of inflammatory cells were found.Under electric microscope,absence of inner arm was found in 1 patient showed,while some cilium mixed together forming a combination was detected in another one.Conclusion KS has the classical triad of pansinusitis,bronchiectasis and situs inversus.Bronchiolitis should be listed as one of the characteristics of KS,which may associate with DPB.