Kartagener's syndrome in Chinese patients

Abstract

The clinical features, ciliary function and ultrastructure in Kartagener's syndrome (KS) (sites inversus, sinusitis and bronchiectasis) have never been systematically studied in Orientals. Only 7 cases (4M) of KS have been diagnosed in our hospitals: mean age of onset/assessment, FEV1/FVC, sputum, ciliary beat frequency were 15.9134.9 yrs, 95.6195.6 %pred, 26.6ml/day and 9.1Hz respectively. HRCT revealed bronchiectasis in the lower lobes in all and middle lobe/lingular in 4 cases. Only 1 case had immotile cilia. Transmission electron microscopy revealed the absence of outer dynein arms in 3 patients. The results of this study suggest that patients with Kartagener's syndrome may have normal ciliary ultrastructure although the absence of dynein arms might carry a poor prognostic value as these patients appear to have more active disease.