Kartagener综合征1例报道并文献复习  

Kartagener syndrome:A case report and review of the literature

作　　者：王娜[1] 薛新颖[1] 薛庆亮[1] 张丽娜[1] 郭丽娜[1] 汪建新[1] 

机构地区：[1]解放军总医院呼吸科,北京100853

出　　处：《军医进修学院学报》2011年第6期659-660,668,共3页Academic Journal of Pla Postgraduate Medical School

摘　　要：目的探讨Kartagener综合征(Kartagener Syndrome,KS)的临床特点、诊断和治疗方法。方法报道1例KS病例,结合文献进行回顾性分析。结果患者女性,16岁,自幼易反复患呼吸道感染,CT示支气管扩张并感染、全内脏转位,经对症治疗后症状好转。KS是一种常染色体遗传性疾病,临床上以咳嗽、咳痰、流脓涕等症状最为常见,CT常提示支气管扩张、内脏转位、鼻窦炎。对症治疗为主要治疗方法。结论 KS是一种少见疾病,临床上极易误诊为"支气管扩张、鼻窦炎",完全型KS的临床诊断主要依据"支气管扩张、内脏转位、鼻窦炎"三联征。如果能做到早期诊断、早期治疗并预防感染等并发症,预后一般良好。Objective To study the clinical features,diagnosis and treatment of Kartagener syndrome（KS）.Methods One case of KS diagnosed in our hospital was retrospectively analyzed and its related literature was reviewed.Results The patient was a 16-year old female with repeated respiratory infection since her childhood.CT showed bronchiectasis accompanying infection and situs perversus.Symptoms of the patient were improved after expectant treatment.KS is a hereditary disease of autosome.Its most common symptoms are cough,expectoration,and nasal discharge.Chest CT scan usually shows bronchiectasis,situs perversus,and nasosinusiti,with expectant treatment as its main therapy.Conclusion KS is a rare disease which can be easily misdiagnosed as bronchiectasis or nasosinusitis.Clinical diagnosis of KS is mainly based on the triad of bronchiectasis,situs perversus and nasosinusitis.Early diagnosis and treatment of KS can prevent its complications and achieve a better prognosis.

关 键 词：KARTAGENER综合征 支气管扩张 鼻窦炎 内脏转位 

分 类 号：R596[医药卫生—内科学]