CYSTIC FIBROSIS IN ADULTS - STUDIES OF PULMONARY FUNCTION AND SOME PHYSICAL PROPERTIES OF BRONCHIAL MUCUS

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Abstract
Pulmonary function studies were performed on 3 men and one woman older than 20 years with long histories of cystic fibrosis, and the flow resistance of their bronchial secretions was measured. Autopsies were performed in 2 of the 4 patients. The functional abnormalities were: airway obstruction, stiffening of the lungs and, in 3 of the 4 patients, increased arterial carbon dioxide tension and reduced arterial oxygen saturation. There were no predominant signs of diffuse fibrotic changes in the lungs. The initial viscosity and the viscosity after shearing were increased in samples of bronchial mucus from the patients. It was concluded that the primary and persistent cause of the respiratory disease in cystic fibrosis is probably the obstruction of the airways secondary to impaired flow of abnormal bronchial mucus. The practical conclusion would be to search for methods that could be used for mobilization of the mucus from the airways.

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