Elsevier

Human Pathology

Volume 21, Issue 6, June 1990, Pages 640-647
Human Pathology

Original contribution
Ultrastructure of airway epithelial cell membranes among patients with cystic fibrosis

https://doi.org/10.1016/S0046-8177(96)90011-8Get rights and content

Abstract

Alterations in transepithelial ion fluxes are a primary pathophysiologic feature in cystic fibrosis (CF). Chronic respiratory infections and host responses are secondary aggravating pulmonary complications of this disease. In the present study, the application of the freeze-fracture technique to samples of nasal and tracheal epithelium from patients with CF has provided a perspective of large areas of cell membrane for the evaluation of possible structural correlates to the pathophysiology of this disease. A variety of aberrant configurations in stranding pattern and disorganization of the epithelial tight junctional complexes in CF airway epithelium are described. Additionally, examination of ciliary membranes revealed the presence of compound cilia and dysmorphology of ciliary necklace configuration. These features are thought to represent acquired structural lesions possibly derived from chronic infection and/or host responses which may further exacerbate abnormal ion transport properties and decrements of ciliary function that appear to be associated with the airway epithelium of individuals with CF.

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    This study was supported in part by National Institutes of Health SCOR Grant HL19171 and PPG Grant HL34322, Environmental Protection Agency Cooperative Agreement CR-807392, and Cystic Fibrosis Foundation RRDP.

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