title: Lung function comparison between two decades in cystic fibrosis children: A single centre study.
abstract: OBJECTIVE
  The purpose of this study was to compare two cohorts of cystic fibrosis (CF) patients born and treated in two different decades, diagnosed through a CF neonatal screening program.
  
  
  METHODS
  We compared pulmonary function decline from 10 to 15 years of age in patients with cystic fibrosis born between 1979 and 1984 (Cohort 1) and between 1991 and 1996 (Cohort 2). Forced expiratory volume in 1 sec (FEV1%) and forced expiratory flow from 25% to 75% (FEF 25-75%) were analyzed by a linear mixed model approach. The differences between the two cohorts were estimated and the overall cohort effect was tested.
  
  
  RESULTS
  Ninety-two patients (51 males, 41 females) fulfilled the selection criteria. Pancreatic insufficiency and CF related diabetes were present in 91% and 20% of patients, respectively. The mean absolute decrement of FEV1% was 9.2 (standard deviation [SD] 11.2) in Cohort 1 and 0.6 (SD 10.4) in Cohort 2 (P < 0.001). The mean decrement of FEF 25-75% was 16.3 (SD 19.5) in Cohort 1 and 1.3 (SD 16.8) in Cohort 2 (P < 0.001) and the Pseudomonas aeruginosa (Pa) colonization was 28% and 15% respectively (P = 0.1).
  
  
  CONCLUSIONS
  Our results show that pulmonary function has clearly ameliorated over a decade in young CF patients, in a period during which several significant therapeutic changes have been introduced, such as dornase alfa, tobramycin and hypertonic saline. To our knowledge this is the first study showing a cohort effect in patients diagnosed after neonatal screening.
authors.affiliation: Cystic Fibrosis Center, Verona, Italy.
authors.forename: Gloria
authors.initials: G
authors.lastname: Tridello
authors.affiliation: Cystic Fibrosis Center, Verona, Italy.
authors.forename: Sonia
authors.initials: S
authors.lastname: Volpi
authors.affiliation: Cystic Fibrosis Center, Verona, Italy.
authors.forename: Baroukh M
authors.initials: BM
authors.lastname: Assael
authors.affiliation: Cystic Fibrosis Center, Verona, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Meneghelli
authors.affiliation: Cystic Fibrosis Center, Verona, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Passiu
authors.affiliation: CHIESI FARMACEUTICI Spa, Parma, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Circelli
mesh_terms: D000293:Adolescent; D002648:Child; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D003920:Diabetes Mellitus / Q000453:epidemiology; D019468:Disease Management; D042241:Early Diagnosis; D010188:Exocrine Pancreatic Insufficiency / Q000453:epidemiology; D005260:Female; D005540:Forced Expiratory Flow Rates / Q000502:physiology; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D008297:Male; D015997:Neonatal Screening; D011552:Pseudomonas Infections / Q000453:epidemiology; D012189:Retrospective Studies; D013203:Staphylococcal Infections / Q000453:epidemiology
keywords: Cystic fibrosis; FEV1; adolescence; nebulized antibiotics