完全型Kartagener综合征一例并文献复习  

A case report of complete Kartagener′s syndrome and literature review

作　　者：张玉佩 ZHANG Yupei(Community Medical Office,Liaocheng People's Hospital,Shandong Province,Liaocheng252000,China)

机构地区：[1]山东省聊城市人民医院社区办,山东聊城252000

出　　处：《中国当代医药》2023年第25期150-153,共4页China Modern Medicine

摘　　要：Kartagener综合征(KS)是原发性纤毛不动障碍的一种亚型,主要的临床表现为全内脏转位-支气管扩张,伴有(或不伴有)鼻窦炎或鼻息肉,绝大多数为常染色体隐性遗传,属临床罕见疾病。该病最主要的表现为全内脏转位、支气管扩张、鼻窦炎或鼻息肉,以影像学检查为主要的诊断手段,以CT检查为主。目前,针对该病的主要治疗为对症处理及预防感染。该病发病率极低,多以文献个案报道为主。本文对1例完全型KS患者的临床诊疗资料进行回顾性分析,并结合国内外相关文献报道,总结病因、发病机制、临床表现、治疗及预后,旨在提高对KS的认识,减少疾病的误诊、漏诊,改善诊疗效果及疾病预后,降低疾病负担。Kartagener's syndrome(KS)is a subtype of primary ciliary dyskinesia characterized by the clinical presentation of total visceral translocation-bronchiectasis with(or without)sinusitis or nasal polyps Most of them are autosomal recessive,which is a rare clinical disease.The most common manifestations of the disease were transposition of internal organs,bronchiectasis,sinusitis or nasal polyps.The main diagnostic methods were imaging examination and CT scan.At present,the main treatment for the disease is symptomatic treatment and prevention of infection.The incidence of this disease is very low and most cases are reported in literature.The clinical data of a patient with complete KS were analyzed retrospectively.The etiology,pathogenesis,clinical manifestation,treatment and prognosis were summarized,the purpose of this study is to improve the understanding of KS,reduce the misdiagnosis and missed diagnosis,improve the effect of diagnosis and treatment,and reduce the disease burden.

关 键 词：KARTAGENER综合征 全内脏转位 支气管扩张 鼻窦炎 文献复习 

分 类 号：R563[医药卫生—呼吸系统]