Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis

Objective: To determine the clinical consequences of acquiring Pseudomonas aeruginosa infection during early childhood in children with cystic fibrosi s (CF). Design: Prospective, observational cohort study of 56 children with CF iden tified by newborn screening during 1990-92. Each child underwent an annual bronchial lavage during the first 2 to 3 years of life. Clinical outcome wa s determined at 7 years of age. Results: Paeruginosa infection was diagnosed in 24 (43%) cohort subjects. F our children died before 7 years of age, all of whom had been infected with a multi-resistant, mucoid strain of Paeruginosa (P = .04). In survivors, P aeruginosa infection was associated with significantly increased morbidity as measured by lower National Institutes of Health scores, greater variabil ity in lung function, increased time in the hospital, and higher rates of r ecombinant human deoxyribonuclease therapy (P < .01). In this young CF coho rt, best forced expiratory volume in 1 second was an insensitive measure of increased morbidity. Conclusions: Acquisition of Paeruginosa was common by 7 years of age in thi s CF birth cohort and was associated with increased morbidity and mortality . An improved disease severity score would improve the evaluation and study of early CF lung disease.