Kartagener 증후군 4예

Four Cases of Kartagener's Syndrome

저자들은 Kartagener 증후군의 전형적인 특징인 내장 좌우역전증, 기관지 확장증 및 부비동염과 함께, 각각 폐암, 성인 호흡장애 증후군등을 동반한 2예를 포함한 4예를 경험하였기에 문헌고찰과 함께 이를 보고하는 바이다.

Kartagener's sydrome is an autosomaly inherited recessive condition characterized by situs inversus, bronchiectasis, and chronic sinusitis. And recently it was recognized as a subclass of dyskinetic cilia syndrome which caused by a defect in mucociliary transport owing to immotile or dyskinetic beating of cilia. Electron microsopy of cilia from sperm tails, nasal and bronchial epithelium of patients reveals the partial or complete absence of dynein arms. Our four patients were diagnosed as a Kartagener's syndrome by classic triad. We carried out electron microscopy of cilia of the nasal mucosa. And many other tests were done. One patient had squamous cell carcinoma of the lung, and another one patient revealed features of adult respiratory distress syndrome at admission. All patients improved with conservative therapy such as physiotherapy, bronchodilater, antibiotics except one patient who mechanical ventilation was required. A brief review of literature was made.