title: Association between mucoid Pseudomonas infection and bronchiectasis in children with cystic fibrosis.
abstract: OBJECTIVE
  To correlate the severity of bronchiectasis in children with cystic fibrosis with clinical and microbiologic variables in order to clarify risk factors for the development of irreversible lung disease.
  
  
  METHODS
  After institutional review board approval and parental informed consents were obtained, a HIPAA-compliant longitudinal epidemiologic evaluation was performed in patients with cystic fibrosis who were enrolled in the Wisconsin trial of newborn screening from 1985 to 2009. Thin-section chest computed tomography (CT) was used in a prospective cross-sectional design to study patients ranging in age from 6.6 to 17.6 years (mean, 11.5 years). Thin-section CT scores were determined objectively on coded images by multiple raters in a standardized fashion. Microbiologic data were obtained by means of culture of respiratory secretions by using methods for differentiation of Pseudomonas aeruginosa (PA) as either nonmucoid or mucoid.
  
  
  RESULTS
  Eighty-three percent of patients (68 of 82) showed bronchiectasis of varying severity. Of 12 potential risk factors, only respiratory infection with mucoid PA correlated significantly with bronchiectasis (P = .041).
  
  
  CONCLUSIONS
  The severity of bronchiectasis in children with cystic fibrosis is significantly related to respiratory infection with mucoid PA; attempts to prevent bronchiectasis should include reducing exposure to and early eradication of PA.
authors.affiliation: Department of Pediatrics, University of Wisconsin, Madison, Wis, USA.
authors.forename: Philip M
authors.initials: PM
authors.lastname: Farrell
authors.forename: Jannette
authors.initials: J
authors.lastname: Collins
authors.forename: Lynn S
authors.initials: LS
authors.lastname: Broderick
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Rock
authors.forename: Zhanhai
authors.initials: Z
authors.lastname: Li
authors.forename: Michael R
authors.initials: MR
authors.lastname: Kosorok
authors.forename: Anita
authors.initials: A
authors.lastname: Laxova
authors.forename: William M
authors.initials: WM
authors.lastname: Gershan
authors.forename: Alan S
authors.initials: AS
authors.lastname: Brody
mesh_terms: D000293:Adolescent; D001987:Bronchiectasis / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D002648:Child; D015897:Comorbidity; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008297:Male; D015995:Prevalence; D011552:Pseudomonas Infections / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D018570:Risk Assessment; D012307:Risk Factors; D014057:Tomography, X-Ray Computed / Q000706:statistics & numerical data*; D014481:United States / Q000453:epidemiology
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