Severe respiratory disease in infants with cystic fibrosis.

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, 36.5 mm Hg). Tracheal cultures showed 
a predominance of gram-negative microorganisms with Pseudomonas aeruginosa, Klebsiella 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 screen 
test for CF at birth. 