CAJ | 학술논문

目的对比分析中外儿童Kartagener综合征的诊断特点.方法回顾性分析山东大学附属省立医院通过电镜确诊的4例Kartagener综合征患儿的临床资料,并复习相关文献,对比分析中国与国外对该病的临床表现、辅助检查及诊断特点.结果 4例患儿临床表现均有反复排痰性咳嗽、鼻窦炎的症状,体格检查发现心尖搏动均位于右侧,影像学检查均有鼻窦炎、右位心、胸腹腔脏器反位及支气管扩张的表现,支气管镜下可见气道结构左右转位,进一步行支气管黏膜活检,电镜下均表现为纤毛结构的异常,据此4例患儿均确诊为Kartagener综合征.以"Kartagener综合征"和"儿童"为检索词在中国知网及PubMed上检索相关病例报道,检索到儿童相关病例报道中国55例、国外61例.诊断年龄国内为(9.16±3.67)岁,国外(7.07±4.92)岁(=2.642,P＜0.01).所描述的主要临床表现为反复排痰性咳嗽(中国54例,国外35例,x2=26.966,P＜0.01),鼻窦炎或鼻息肉(中国49例,国外33例,x2=17.093,P＜0.01),反复肺部感染(中国33例,国外32例),反复喘息(中国18例,国外24例)和中耳炎(中国9例,国外10例).鼻窦影像学检查示上颌窦炎(中国43例/48例、国外8例/10例).胸腹部影像学检查示全部右位心(中国54例、国外34例),全内脏转位(中国50例/54例、国外33例/34例),支气管扩张(中国50例/54例,国外17例/34例,x2=20.832,P＜0.01).纤毛超微结构检查示动力蛋白臂变短或缺失(中国6例/6例、国外25例/27例).结论中外Kartagener综合征临床均表现为反复上下呼吸道感染并伴右位心或其他内脏转位,国外对Kartagener综合征的诊断更多是通过电镜下观察到纤毛超微结构的异常,国内对该病的诊断主要根据其典型的临床表现.
목적 대비분석중외인동Kartagener종합정적진단특점.방법 회고성분석산동대학부속성립의원통과전경학진적4례Kartagener종합정환인적림상자료,병복습상관문헌,대비분석중국여국외대해병적림상표현、보조검사급진단특점.결과 4례환인림상표현균유반복배담성해수、비두염적증상,체격검사발현심첨박동균위우우측,영상학검사균유비두염、우위심、흉복강장기반위급지기관확장적표현,지기관경하가견기도결구좌우전위,진일보행지기관점막활검,전경하균표현위섬모결구적이상,거차4례환인균학진위Kartagener종합정.이"Kartagener종합정"화"인동"위검색사재중국지망급PubMed상검색상관병례보도,검색도인동상관병례보도중국55례、국외61례.진단년령국내위(9.16±3.67)세,국외(7.07±4.92)세(=2.642,P＜0.01).소묘술적주요림상표현위반복배담성해수(중국54례,국외35례,x2=26.966,P＜0.01),비두염혹비식육(중국49례,국외33례,x2=17.093,P＜0.01),반복폐부감염(중국33례,국외32례),반복천식(중국18례,국외24례)화중이염(중국9례,국외10례).비두영상학검사시상합두염(중국43례/48례、국외8례/10례).흉복부영상학검사시전부우위심(중국54례、국외34례),전내장전위(중국50례/54례、국외33례/34례),지기관확장(중국50례/54례,국외17례/34례,x2=20.832,P＜0.01).섬모초미결구검사시동력단백비변단혹결실(중국6례/6례、국외25례/27례).결론 중외Kartagener종합정림상균표현위반복상하호흡도감염병반우위심혹기타내장전위,국외대Kartagener종합정적진단경다시통과전경하관찰도섬모초미결구적이상,국내대해병적진단주요근거기전형적림상표현.
Objective To analyze the clinical characteristic of Kartagener syndrome in Chinese and foreign children.Method Four cases of Kartagener syndrome diagnosed in our hospital were analyzed with literature review.The differences between Chinese and foreign children in clinical manifestations and diagnosis were compared.Result All of the cases had the following clinical manifestations: recurrent productive cough, nasosinusitis, dextrocardia, total situs inversus and bronchiectasia.Situs inversus of the airway structure was visible under the bronchoscope, and electron microscopy of the respiratory mucosa showed an abnormal ciliary ultrastructure.By using "Kartagener syndrome" and "child" as the key words,reports on 55 Chinese cases and 61 foreign cases in children were retrieved from CNKI and PubMed databases.The average age of diagnosis was 9.16 ± 3.67 years in China, which was significantly later than 7.07 ± 4.92 years in foreign countries (t =2.642, P ＜ 0.01).The main clinical manifestations were recurrent productive cough, nasosinusitis or rhinopolyp, recurrent pulmonary infection, recurrent wheezing and otitis media.Sinus imaging showed maxillary sinusitis.Dextrocardia, total situs inversus and bronchiectasia were found on thoracic and abdominal CT.The ciliary ultrastructural analysis showed shorter and missing dynein arm (6/6 cases in China and 25/27 cases in foreign).Conclusion The clinical manifestations are recurrent upper and lower respiratory tract infection combined with dextrocardia or other situs inversus in both China and other countries.The diagnosis abroad relied more on abnormal ciliary ultrastructure observed under electron microscopy, but in China mainly relied on its typical clinical manifestations.