This Specialized Center of Research will continue to provide a central supporting structure for the efforts of many investigators concerned with the pulmonary problems of cystic fibrosis and provide a forum for integration and cooperation between their individual projects. The primary topic of the SCOR is being addressed by both clinical and basic research projects. The former are attempting to find better means of patient care and more sensitive indicators of disease progression while the latter are studying the pathologic mechanisms of lung injury and the basic metabolic defect. The specific goals of the individual projects are: -To use a centralized patient data base and analysis system to coordinate patient care and research studies. -To demonstrate the feasibility and medical effectiveness of using automated analysis of home measurements for improving clinical care. -To continue the evolution of a new pulmonary function test for patients of all ages and to use this test to follow normal lung development in young children and disease progression in CF patients. -To investigate the structure and function of mucopolysaccharide molecules in bronchial mucus. -To evaluate a possible mechanism of CF lung destruction by pulmonary macrophages and continue investigations of their metabolic, structural, and phagocytic properties. -To expand upon observations of enhanced metabolic activities of CF monocytes. -To extend the knowledge of the deranged NADH dehydrogenase found in mitochondria from CF subjects as a probably basic defect in CF cells and investigate the possibility of a prenatal test for CF.
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