Primary and Secundary Ciliary Dyskinesia

Abstract

It has recently been shown that patients with Kartagener's triad and also some subjects with similar symptoms, but without situs inversus, have a congenital abnormality of cilia as an explanation for their chronic airway symptoms; this disease has been named “the im-motile-cilia syndrome” or more correctly “primary ciliary dyskinesia”. Studying 27 such patients, we have found daily nose blowings since birth, chronic-recurrent sinus itis, and chronic secretory otitis media highly characteris tic features. The frequency of common colds was not increased, and most patients did not suffer from repeated episodes of acute purulent otitis media. The number of ciliated cells with immotile cilia was increased, but only a single patient had completely immotile cilia; also the de gree of asynchrony within the single ciliated cell was increased. Electron microscopy showed a decreased num ber of dynein arms in some patients, and abnormal ar rangement of microtubules in others. Some patients, how ever, had normal ultrastructure, and this appeared to be associated with a hyperfrequenct beating pattern. At least three subgroups of patients with primary ciliary dyskine sia can be delineated based on the motility-ultrastructure studies. Bacterial infection tends to reduce the number of ciliated cells with motile cilia, and viral infection (com mon cold) gives a very marked and long-lasting reduction in the number of ciliated cells. This may account for some otherwise unexplainable subchronic symptoms from nose and throat.