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Cystic Fibrosis:  Pulmonary Studies in Children, Adolescents, and Young Adults

Patricia S. Zelkowitz, MD; Samuel T. Giammona, MD
Am J Dis Child. 1969;117(5):543-547. doi:10.1001/archpedi.1969.02100030545007.
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THE SURVIVAL of children with cystic fibrosis beyond the newborn period is directly associated with the severity of their pulmonary involvement. In the last decade, the advances in medical care have resulted in the survival of more children with cystic fibrosis into young adulthood.1 This has allowed objective evaluation of the progression of pulmonary involvement in these patients. Cystic fibrosis has been found to be associated with a pattern of airway obstruction,2,3 poor intrapulmonary gas mixing,4,5 increase in residual volume,6,7 loss of lung elasticity,7 and changes in gas diffusion.8

This report is an analysis of the studies of 66 children with cystic fibrosis who have a wide spectrum of pulmonary disease and whose intensity of therapy and years of treatment are variable. Pulmonary function studies were evaluated to determine which tests could be done simply and frequently, yet provide accurate assessments of current pulmonary

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