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目的探讨Kartagener综合征的临床特点、诊断标准,以提高对Kartagener综合征认识。方法 Kartagener综合征1例。患儿,女,10岁余。因反复咳嗽1年,咯血4次入院。回顾性分析该患儿临床资料,检索国内外文献,进行总结分析。结果患儿隐匿起病,主要表现为反复咳嗽,咳脓痰,伴咯血。CT示双肺支扩伴感染,全内脏反位,鼻窦炎。经抗感染对症治疗1周后,临床症状消失出院。检索国内外文献发现,Kartagener综合征以全内脏反位,支气管扩张,副鼻窦炎三联征为诊断标准。治疗以对症治疗为主。结论 Kartagener综合征是一种罕见的常染色体隐性遗传病,加强对该病的认识,有利于提高对该病的诊断率,减少误诊率。对该病的致病基因、机制的进一步研究,可能提供更好的诊断和治疗方法。
Objective To investigate the clinical features and diagnostic criteria of Kartagener syndrome so as to improve the understanding of Kartagener’s syndrome. Methods Kartagener syndrome in 1 case. Children, women, more than 10 years old. Due to repeated cough for 1 year, 4 cases of hemoptysis admitted to hospital. Retrospective analysis of the clinical data of children, retrieve the literature at home and abroad, for a summary analysis. Results Children concealed onset, mainly for repeated cough, cough purulent sputum, with hemoptysis. CT showed double pulmonary branch expansion with infection, the entire visceral anti-bit, sinusitis. After anti-infective symptomatic treatment for 1 week, the clinical symptoms disappeared. Retrieval of domestic and foreign literature found that Kartagener syndrome with total visceral anti-bit, bronchiectasis, sinusitis triad as the diagnostic criteria. The main symptomatic treatment. Conclusion Kartagener syndrome is a rare autosomal recessive disease, to enhance awareness of the disease, help to improve the diagnosis of the disease and reduce the misdiagnosis rate. Further study of the causative genes and mechanisms of the disease may provide better diagnostic and therapeutic approaches.