Cystic Fibrosis of the Pancreas—A Comparison of the Pulmonary Manifestations in Children and Young Adults

Clinical Conference in Pulmonary Disease from Northwestern University Medical Center, Chicago

¹ Chief, Pulmonary Function Laboratory, Chicago Wesley Memorial Hospital Assistant Professor of Medicine
² Chief, Pulmonary Disease Section, Ernest S. Bazley Professor of Medicine
³ Director of Clinical Study Center, Children's Memorial Hospital; Associate in Pediatrics
⁴ Chief, Cystic Fibrosis Clinic; Assistant Professor of Pediatrics
⁵ Resident in Medicine, Chicago Wesley Memorial Hospital

Dr. Cugell: We've heard an illuminating discussion of cystic fibrosis of the pancreas with emphasis upon the pulmonary manifestations of this disease. Recurrent pulmonary infection with eventual respiratory insufficiency is the major incapacitating feature of this disease in young adults whereas, in infancy and childhood, gastrointestinal symptoms are more commonplace. A sweat chloride concentration in excess of 80 mEq/L is diagnostic of this disease in adults and concentrations of 60 to 80 mEq/L are borderline. Concentrations above 60 mEq/L are diagnostic in children with values of 40 to 60 mEq/L being of questionable significance. By appropriately combining physical methods to promote bronchial drainage, high humidity aerosol treatments and antibiotics, the frequent pulmonary infections and consequent tissue destruction can be minimized but not entirely prevented. Incomplete resolution of pulmonary infections with failure of the roentgenogram to revert to normal is indicative of a poor outlook. If these patients reach the teens and young adult years they may be troubled by numerous economic, social and psychologic problems. For infants and young children comparable anxieties exist but in their parents. Meticulous attention to proper treatment principles now enables many youngsters to reach adulthood, but once pulmonary insufficiency commences their life expectancy is exceedingly shortened.