Bronchiolitis in Kartagener's syndrome

The association of diffuse bronchiolitis in patients with Kartagener's synd rome (KS) has not been reported previously. The aim of this study was to pr esent the morphological characteristics of bronchiolitis in patients with K S. Eight patients (four males, four females; mean age 37.9+/-18.7 yrs), clinic ally diagnosed as KS with the classical triad of chronic pansinusitis, bron chiectasis and situs inversus with dextrocardia, were evaluated. Routine chest radiography showed bronchiectasis and dextrocardia in all pat ients. Chest computed tomography (CT) showed diffuse centrilobular small no dules up to 2 mm in diameter throughout both lungs in six out of eight pati ents. Pulmonary function tests revealed marked obstructive impairment in ai l patients (forced expiratory volume in one second 57.0+/-11.3%, residual v olume/total lung capacity 45.0+/-12.7%, maximum midexpiratory now 0.92+/-0. 72 L.s(-1), forced vital capacity 74.1+/-12.2% (all mean+/-SD)). The examin ation of cilial movement of the bronchus revealed immotility in all of the five patients examined. The ultrastructure showed ciliary dynein arm defect s in all patients. Histopathological examination of lung specimens obtained at autopsy or by video-assisted thoracoscopic surgery showed obliterative thickening of the walls of the membranous bronchioli with infiltration of l ymphocytes, plasma cells and neutrophils, but most of the distal respirator y bronchioli were spared and alveolar spaces were overinflated. Pathologica lly, the diffuse centrilobular small nodules on the chest CT mainly corresp onded to membranous bronchiolitis. This is the first report demonstrating that the association of diffuse bron chiolitis might be one of the characteristic features of the lung in Kartag ener's syndrome.