title: Is the type of chronic pulmonary infection a determinant of lung function outcomes in adult patients with cystic fibrosis?
abstract: OBJECTIVE
  Lung function abnormalities are the main factors responsible for the high mortality of cystic fibrosis (CF) patients. It is not yet clear whether Burkholderia cepacia infection causes more pronounced loss of lung function than pseudomonas aeruginosa infection. Our primary objective was to compare the lung function of adult CF patients with different chronic pulmonary infections. Our second objective was to compare the microbiology using patients' genetic status.
  
  
  METHODS
  Fifty-two adult CF patients were divided into 3 groups according to their chronic pulmonary infection profile. All subjects underwent clinical evaluation, pulmonary function tests (PFT) and genetic analysis.
  
  
  RESULTS
  The PFT parameters of chronically infected patients were significantly different from those of subjects without pulmonary infection (p < 0.0001). FVC was significantly more altered in patients infected with B. cepacia complex (p < 0.0001); in contrast, FEF25-75% was significantly more altered in patients with P. aeruginosa infection (p < 0.0001). In the groups with chronic P. aeruginosa infection and chronic B. cepacia complex infection, 58.1% and 10% of patients were homozygous for AF508, respectively. In addition to chronic infections, pancreatic insufficiency was also associated with lung function deterioration.
  
  
  CONCLUSIONS
  Chronic pulmonary infection and pancreatic insufficiency are critical processes in lung function deterioration in adult CF patients. Although chronic B. cepacia complex infection causes a more pronounced lung volume reduction, chronic P. aeruginosa infection causes a more pronounced obstruction of small airways. Our results also suggest that deltaF508-homozygous patients are more susceptible to chronic P. aeruginosa infection.
authors: Laboratory of Respiratory Physiology, University of the State of Rio de Janeiro, and Rehabilitation Science Graduate Program, Augusto Motta University, Rio de Janeiro. phel.lop@uol.com.br
authors: A J
authors: AJ
authors: Lopes
authors: T T
authors: TT
authors: Mafort
authors: A
authors: A
authors: de Sá Ferreira
authors: M C
authors: MC
authors: Santos de Castro
authors: M
authors: M
authors: Cássia de Firmida
authors: E
authors: E
authors: de Andrade Marques
mesh_terms: D000328:Adult; D001419:Bacteria / Q000302:isolation & purification; D002908:Chronic Disease; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000382:microbiology / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D012141:Respiratory Tract Infections / Q000382:microbiology / Q000503:physiopathology*