Severe Respiratory Disease in Infants With Cystic Fibrosis

Abstract

The diagnosis, management and prognosis of 17 infants with cystic fibrosis (CF) and severe respiratory disease were reviewed for the period 1968 to 1972. The clinical course of these infants was characterized by a bronchiolitis-like syndrome with failure to thrive and malnutrition. Arterial blood gases demonstrated marked hypoxemia (mean Pao2, 37.5 mm Hg). Tracheal cultures showed a predominance of gram-negative microorganisms with Pseudomonas aeruginosa, Kiebsiella pneumoniae and Escherichia coli predominating. Corticosteroids were used in all patients. Despite vigorous therapy including antibiotics there was a 60% mortality. A delay in the diagnosis of CF from the onset if respiratory symptoms with a mean of six weeks was considered an important factor affecting survival. This data supports the need for developing a reliable screening test for CF at birth.