Four Cases of Kartagener's Syndrome / 결핵

ABSTRACT

Kartagener's syndrome 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.