title: Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Pediatrics, University of Wisconsin, Madison 53705-2221, USA.
authors.forename: Zhanhai
authors.initials: Z
authors.lastname: Li
authors.forename: Michael R
authors.initials: MR
authors.lastname: Kosorok
authors.forename: Philip M
authors.initials: PM
authors.lastname: Farrell
authors.forename: Anita
authors.initials: A
authors.lastname: Laxova
authors.forename: Susan E H
authors.initials: SE
authors.lastname: West
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Green
authors.forename: Jannette
authors.initials: J
authors.lastname: Collins
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Rock
authors.forename: Mark L
authors.initials: ML
authors.lastname: Splaingard
mesh_terms: D000293:Adolescent; D000907:Antibodies, Bacterial / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D009017:Morbidity; D016016:Proportional Hazards Models; D011446:Prospective Studies; D011552:Pseudomonas Infections / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D011550:Pseudomonas aeruginosa / Q000235:genetics / Q000502:physiology*; D014774:Virulence
chemical_list: D000907:Antibodies, Bacterial