作者：李亚明，张进召，刁鑫

单位：

710077 陕西省西安市，西安医学院第一附属医院呼吸与危重症医学科；通信作者：张进召，E-mail：zhangjinzhao2006@sina.com

Units：

Department of Pulmonary and Critical Care Medicine，the First Affiliated Hospital of Xi'an Medical University，Xi'an710077，China；Corresponding author：ZHANG Jinzhao，E-mail：zhangjinzhao2006@sina.com

关键词：

卡塔格内综合征；纤毛运动障碍；支气管扩张症；鼻窦炎；内脏异位综合征；病例报告；历史文献

Keywords：

Kartagener syndrome；Ciliary motility disorders；Bronchiectasis；Sinusitis；Heterotaxy syndrome；Case reports；Historical article

CLC：

R 56

DOI：

DOI：10.3969/j.issn.1008-5971.2020.02.020

Funds：

摘要：

Kartagener 综合征是一种临床罕见的常染色体隐性遗传性疾病，发病率极低，主要表现为支气管扩张、慢性鼻窦炎、内脏反位三联征，电镜下纤毛超微结构联合光镜下纤毛功能检查是目前临床诊断 Kartagener 综合征的“金标准”。本文报道了两例完全型 Kartagener 综合征患者并进行了文献复习，为进一步提高临床对该病的诊治水平提供参考。

Abstract：

Kartagener syndrome，as one kind of autosomal recessive hereditary diseases with very low morbidity，is rare on clinic. Patients with Kartagener syndrome mainly performed as triad syndrome of bronchiectasia，chronic nasosinusitisand situs inversus，but ultrastructure of cilia under electron microscope combined with cilia function examined by lightmicroscope is the "golden standard" at present. This paper reported two patients with complete Kartagener syndrome and reviewedpertinent literatures，in order to provide a reference for improving the clinical diagnostic and therapeutic level.

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