Kartagener综合征11例临床分析  

The clinical analysis of 11 cases of patients with Kartagener syndrome

作　　者：郭涛[1] 杨贵芳[1] 彭静[1] 黄钰财[1] 柴湘平[1] 

机构地区：[1]中南大学湘雅二医院急诊科/急诊疑难病研究所,长沙410011

出　　处：《疑难病杂志》2016年第6期637-640,共4页Chinese Journal of Difficult and Complicated Cases

基　　金：国家自然科学基金资助项目(81471896)

摘　　要：目的总结Kartagener综合征(KS)患者的临床特点、诊断及治疗方法,以减少KS的误诊、误治。方法回顾分析2011年7月—2015年6月中南大学湘雅二医院急诊科/急诊疑难病研究所收治的KS患者11例的临床资料、影像资料及随访数据。结果 (1)KS患者发病年龄较早,发病具有遗传倾向。(2)咳嗽、咯血、气促是KS综合征就诊的主要症状。(3)KS患者主要的支气管扩张部位为左下肺、双下肺,心脏CTA提示部分KS患者合并心脏结构异常。(4)预防感染、及时控制感染是治疗KS综合征的关键,若患者存在难治性鼻窦炎或合并心脏结构异常,应尽早手术治疗。结论 KS是一种罕见的具有遗传倾向的疾病,预防和及时控制感染是治疗KS的关键,合并心脏结构异常者应尽早手术。Objective To summarize the clinical features,the diagnosis and treatment of Kartagener syndrome（KS）,so as to avoid misdiagnosis and mistreatment.Methods Eleven KS patients treated in our hospital from July 2011 to June 2015 were enrolled,and their clinical data,imaging data and follow-up data were analyzed retrospectively.Results（1）The onset age of KS patients is earlier,and the incidence of the disease has a hcredilary inclination.（2） Cough,hemoptysis and short breath are the main symptoms of KS patients.（3） Bronchiectasis parts of KS patients are the lower left lung,double lung,and heart CTA indicates that some KS patients complicated with cardiac structural abnormalities.（4） Prevention of infection,infection control in time is the key lo treating KS syndrome,if the patients combined with refractory sinusitis or complicated with cardiac structural abnormalities,should he given surgical treatment as early as possible.Conclusion KS is a kind of rare disease,which has a hereditary inclination,prevention,infection control in time is the key to treating KS syndrome,and patients complicated with cardiac structural abnormalities should be given surgical treatment as early as possible.

关 键 词：KARTAGENER综合征 支气管扩张 鼻窦炎 右位心 

分 类 号：R596.1[医药卫生—内科学]