Respir Case Rep. 2014; 3(1): 56-59 | DOI: 10.5505/respircase.2014.15238

Kartagener Sendromu ve Primer Silier Diskinezi: Ýki Kardeþ Olgu

Jülide Çeldir Emre¹, Ümit Aksoy¹, Ayþegül Baysak², Adnan Tolga Öz², Hakan Borand³, Feza Bacakoðlu^4
1Turgutlu Devlet Hastanesi Göðüs Hastalýklarý Kliniði, Manisa
²Ýzmir Üniversitesi Týp Fakültesi, Göðüs Hastalýklarý Anabilim Dalý, Ýzmir
³Turgutlu Devlet Hastanesi,enfeksiyon Hastalýklarý Ve Klinik Mikrobiyoloji,manisa
⁴Department Of Chest Diseases, Ege Universitey School Of Medicine, Izmir, Turkey

Primer silier diskinezi otozomal resesif geçiþli, silier yapý ve fonksiyonunda anormalliklerle giden ve 20000 canlý doðumda bir görülen nadir bir hastalýktýr. Bu hastalýk grubunun %50’sini bronþektazi, kronik sinüzit ve komplet situs inversus kliniðini içeren Kartagener Sendromu oluþturur. Genç olgularýmýzda, kronik üst ve alt solunum yolu enfeksiyonu bulgularý olanlarda olasý bir taný olarak akýlda tutulmalýdýr. Bu yazýda, Kartagener Sendromlu ve primer siliyer diskinezili iki olgu klinik ve radyolojik bulgularý ile sunuldu ve ilgili literatür tartýþýldý.

Anahtar Kelimeler: Kartagener sendromu, Primer silier diskinezi, Bronþektazi

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Kartagener’s Syndrome and Primary Ciliary Dyskinesia: two cases in siblings

Jülide Çeldir Emre¹, Ümit Aksoy¹, Ayþegül Baysak², Adnan Tolga Öz², Hakan Borand³, Feza Bacakoðlu^4
1Department Of Chest Diseases, Turgutlu State Hospital, Manisa, Turkey
²Department Of Chest Diseases, Izmir University School Of Medicine, Izmir, Turkey
³Department Of Infectious Diseases And Clinical Microbiology,turgutlu State Hospital, Manisa, Turkey
⁴Ege Üniversitesi Týp Fakültesi, Göðüs Hastalýklarý Anabilim Dalý, Ýzmir

Primary ciliary dyskinesia (PCD) is a genetically, autosomal-recessive disorder that is caused by abnormal ciliary ultrastructure and function and is a fairly rare condition with a prevalence of 1 in 20,000 live births. Fifty percent of PCD is Kartagener’s syndrome, characterized by bronchiectasis, sinusitis, and complete situs inversus. In conclusion, primary ciliary dyskinesia and Kartegener's syndrome should be considered as a possible diagnosis in young patients with lower and upper respiratory tract infections. Clinical and radiological features of the two cases are presented here. These findings and the related literature are discussed.

Keywords: Kartagener's syndrome, primary ciliar dyskinesia, bronchiectasis

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