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Zhanhai Li, PhD; Michael R. Kosorok, PhD; Philip M. Farrell, MD, PhD; Anita Laxova, BS; Susan E. H. West, PhD; Christopher G. Green, MD; Jannette Collins, MD; Michael J. Rock, MD; Mark L. Splaingard, MD

JAMA. 2005;293:581-588.

Context  Although Pseudomonas aeruginosa is the most common virulent respiratory pathogen in cystic fibrosis (CF), the longitudinal development of P aeruginosa infection and its effect on antibody responses and lung disease progression in children with CF remain unclear.

Objective  To prospectively examine the epidemiology of P aeruginosa infection and its impact on CF pulmonary morbidity.

Design, Setting, and Patients  We prospectively evaluated 56 CF patients at 2 CF centers in Madison and Milwaukee, Wis, from birth up to age 16 years between April 15, 1985, and April 15, 2004, with diagnoses made through the Wisconsin CF Neonatal Screening Project.

Main Outcome Measures  Timing of nonmucoid P aeruginosa and mucoid P aeruginosa acquisition was assessed by first positive result. Longitudinal development from no P aeruginosa to nonmucoid P aeruginosa and from nonmucoid P aeruginosa to mucoid P aeruginosa was examined. Outcome measurements included antibody titers, respiratory symptoms, quantitative chest radiography, and pulmonary function tests.

Results  Sixteen patients (29%) acquired nonmucoid P aeruginosa in the first 6 months of life. The age-specific prevalence of mucoid P aeruginosa increased markedly from age 4 to 16 years. Nonmucoid and mucoid P aeruginosa were acquired at median ages of 1.0 and 13.0 years, respectively. In contrast with the short transition time from no P aeruginosa to nonmucoid P aeruginosa, the transition time from nonmucoid to mucoid P aeruginosa was relatively long (median, 10.9 years) and could be slightly extended by brief/low anti–P aeruginosa antibiotic treatment. Antibody titers increased with both transitions, but the deterioration in cough scores, chest radiograph scores, and pulmonary function correlated best with transition from nonmucoid to mucoid P aeruginosa.

Conclusions  Early prevention and detection of nonmucoid and mucoid P aeruginosa are critical because of early acquisition and prevalence. There is a window of opportunity for suppression and possible eradication (by aggressive anti–P aeruginosa treatment) of initial nonmucoid P aeruginosa. Mucoid P aeruginosa plays a much greater role in CF lung disease progression than nonmucoid P aeruginosa. Antibody titers, cough scores, and chest radiographs are early signs of nonmucoid P aeruginosa and especially mucoid P aeruginosa stages.

Author Affiliations: Departments of Pediatrics (Drs Li, Kosorok, Farrell, Green, and Rock and Ms Laxova), Biostatistics/Medical Informatics (Drs Li and Kosorok), Pathobiological Sciences (Dr West), and Radiology (Dr Collins), University of Wisconsin, Madison; and Department of Pediatrics, Medical College of Wisconsin, Milwaukee (Dr Splaingard).

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