Ciliary dyskinesia with normal ultrastructure.

There is increasing awareness of the association between primary ciliary abnormalities and bronchiectasis. The most frequently recognised is that between dynein deficient cilia and Kartageneres syndrome.' Afzelius, however, predicted that without embryonic ciliary movement the rotation of the archenteron would be random and that in half of the cases of the "immotile cilia syndrome" there would be a normal cardiac situs.2 In recent years other primary structural abnormalities of cilia have been described in association with lower respiratory tract infection-namely absence of radial spokes3 and transposition of the microtubules.4 The finding that some individuals with Kartagener's syndrome have normal ciliary ultrastructureS6 implies that either the association of the clinical syndrome with abnormal cilia does not always hold or that functional abnormalities of cilia may occur in the absence of ultrastructural defects. No observations of ciliary motility have been reported on patients with Kartageneres syndrome and normal ciliary ultrastructure. With Kartagener's syndrome the presence of ciliary dysfunction is readily suspected, but in patients with ciliary dyskinesia and normal situs (who also present with symptoms of sinusitis and bronchiectasis or recurrent chest infections) a functional abnormality may not be recognised unless tests of ciliary motility are performed. We report two cases of ciliary dyskinesia and associated clinical abnormalities but normal ciliary ultrastructure.