Kartagener’s综合征伴泛细支气管炎临床病理分析

Clinical and pathological features of Kartagener's syndrome complicated with diffuse panbronchiolitis

目的探讨Kartagener’s综合征(KS)合并泛细支气管炎的临床病理学特征。方法分析8例Kartagener’s综合征合并泛细支气管炎患者的临床资料和组织形态特点。结果Kart-agener’s综合征是一种罕见的遗传性疾病,它的特点是支气管扩张症、副鼻窦炎以及内脏转位。以呼吸道为首发症状,常合并弥漫性泛细支气管炎(DPB)。病理形态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例透射电镜检查1例为纤毛的内侧臂缺失,1例纤毛畸形,融合成团。结论Kartagener’s综合征合并泛细支气管炎值得引起临床注意,影像及肺活检有助于对本病的诊断。

Objective To explore the clinical and pathological features of Kartagener＇ s syndrome （KS）. Methods The clinical and pathological features of eight patients with Kartagener＇ s syndrome were studied retrospectively. Results Kartagener＇ s syndrome is a rare inherited disease characterized by situs inversus, bronchiectasis ,and chronic sinusitis. Respiratory passage is the initial syndrome and diffuse panbronchiolitis （DPB） is often complicated. Histopathological examination showed thickening of the walls of the terminal bronchioles and respiratory bronchioles with infiltration of inflammatory cells. Under electric microscope, one case showed absence of inner arm and another showed some cilium mixed together, forming a combination. Conclusions Kartagener＇ s syndrome has certain clinical and pathological features. His- topathological examination of lung specimens obtained by open lung biopsy or video-assisted thoracoscopic surgerys is very necessary for the diagnosis.