title: Influence of inflammatory response, infection, and pulmonary function in cystic fibrosis.
abstract: OBJECTIVE
  Recurrent infections and activation of the inflammatory response affect the prognosis of cystic fibrosis (CF). We investigated the relationship between inflammatory response, infection, and pulmonary function in CF.
  
  
  METHODS
  A clinical-cross-sectional study was conducted with 86 subjects: control group (CG, n=31, the same age and sex of the CF group), and CF group (CFG, n=55, age: 1-16 years), further distributed into CFG negative or positive bacteriology (CFGB(-)/CFGB(+)), and CFG negative or positive Pseudomonas aeruginosa (CFGPa(-)/CFGPa(+)). Using the Wald test, multiple linear regression (95% confidence interval) was performed between CG and CFG, and between CG and each of the CF subgroups (CFGB(-)/CFGB(+) and CFGPa(-)/CFGPa(+)). The inflammatory markers evaluated were myeloperoxidase (MPO), adenosine deaminase (ADA) activities, interleukin-1beta (IL-1β), tumor necrosis factor-alpha (TNF-α), C-reactive protein (CRP), nitric oxide metabolites (NOx) levels, and total and differential leukocyte counts.
  
  
  RESULTS
  After adjusting for sex and age, CFG compared to CG revealed an increase of MPO, IL-1β (P<0.001 in all subgroups), and CRP: CFG (P=0.002), CFGB(-) (P=0.007), CFGB(+) (P=0.009), CFGPa(-) (P=0.004) and CFGPa(+) (P=0.020). NOx (P=0.001, P<0.001), leukocytes (P=0.002, P=0.001), and neutrophils (P=0.003, P<0.001) were increased in CFGB(+) and CFGPa(+), respectively. A negative correlation between FEV1 and leukocytes (P=0.008) and FEV1 and neutrophils (P=0.031) resulted in CFG.
  
  
  CONCLUSIONS
  The inflammatory response characterized by the increase of MPO, IL-1β, and CRP is determinant for CF. Also leukocytosis due to neutrophilia determines the pulmonary function deficiency in this disease.
authors.affiliation: Graduate Program in Nutrition, Federal University of Santa Catarina, Brazil.
authors.forename: Leticia Cristina Radin
authors.initials: LC
authors.lastname: Pereira
authors.affiliation: Department of Nutrition and Graduate Program in Nutrition, Federal University of Santa Catarina, Brazil. Electronic address: emilia.moreira@ufsc.br.
authors.forename: Emilia Addison Machado
authors.initials: EA
authors.lastname: Moreira
authors.affiliation: Graduate Program in Nutrition, Federal University of Santa Catarina, Brazil.
authors.forename: Gabriela Datsch
authors.initials: GD
authors.lastname: Bennemann
authors.affiliation: Department of Nutrition and Graduate Program in Nutrition, Federal University of Santa Catarina, Brazil.
authors.forename: Yara Maria Franco
authors.initials: YM
authors.lastname: Moreno
authors.affiliation: Graduate Program in Medical Science, Federal University of Santa Catarina, Brazil.
authors.forename: Ziliani da Silva
authors.initials: Zda S
authors.lastname: Buss
authors.affiliation: Joana de Gusmão Children's Hospital, Brazil.
authors.forename: Eliana
authors.initials: E
authors.lastname: Barbosa
authors.affiliation: Joana de Gusmão Children's Hospital, Brazil.
authors.forename: Norberto
authors.initials: N
authors.lastname: Ludwig-Neto
authors.affiliation: Department of Ecology and Zoology, Federal University of Santa Catarina, Brazil.
authors.forename: Danilo
authors.initials: D
authors.lastname: Wilhelm Filho
authors.affiliation: Department of Clinical Analysis, Federal University of Santa Catarina, Brazil.
authors.forename: Tânia Silvia
authors.initials: TS
authors.lastname: Fröde
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000382:microbiology; D005260:Female; D006801:Humans; D007223:Infant; D008168:Lung / Q000276:immunology / Q000382:microbiology; D008297:Male; D011014:Pneumonia / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000382:microbiology; D011552:Pseudomonas Infections / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D011550:Pseudomonas aeruginosa / Q000276:immunology* / Q000302:isolation & purification
keywords: Adolescents; Children; Cystic fibrosis; Infection; Inflammation; Pulmonary function