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1218 CILIARY ULTRASTRUCTURE AND NASAL MUCOSAL HISTOLOGY IN CYSTIC FIBROSIS

Mucociliary activity is defective in patients with cystic fibrosis (CF) but the ultrastructure of cilia in CF has not been investigated previously. Since defects in ciliary ultrastructure have been reported in another chronic pulmonary disease (NEJM 297,1,1,1977) the ultrastructure of cilia from patients with CF was studied. Nasal and tracheal mucosa was biopsied in 10 CF patients under direct visualization at the time of bronchial lavage and tissue was fixed for electron microscopy in the usual manner. Nasal mucosal biopsies from healthy individuals and from asthmatics on aerosol therapy, matched for age, were also obtained. The architecture of CF cilia seen in cross section was normal (9 + 2 microtubular arrangement, dynein side arms, spokes, and ciliary orientation). However, the mucosal architecture was grossly distorted. The mucosal surface was fragmented with thickened submucosa, increased goblet cells, fewer ciliated cells and sparse cilia. The picture is that of a traumatized epithelium. Further studies are required to determine whether these changes reflect the disease itself or the result of repeated aerosol therapy.