Abnormal cilia in Polynesians with bronchiectasis

Abstract

Bronchial or nasal ciliated epithelium from 13 patients with bronchiectasis (12 Polynesians and 1 European) was examined by electron microscopy. All cilia examined lacked dynein arams. In 2 patients, this was the only abnormality observed, but in the others, various ciliary abnormalities were present, including cilia with missing tubules, extra tubules, and misplaced tubules, as well as compound cilia, intracytoplasmic cilia, vesiculated cilia, and cilia with long winglike folds. In all of these patients pulmonary mucociliary transport rates were either absent or markedly reduced. It is suggested that these abnormalities were due to a mutation or group of mutations, which was the underlying cause of the bronchiectasis.