title: Cystic fibrosis - Comparison between patients in paediatric and adult age.
abstract: Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Although most cases are diagnosed in childhood, diagnosis in adults is apparently increasing.
  
  
  OBJECTIVE
  Evaluate the adult population with CF, comparing patients who were diagnosed before and after 18 years of age.
  
  
  METHODS
  Retrospective analysis of patients followed in three main medical centres in Portugal in 2012. Comparison of two groups: G1 - patients diagnosed at <18 years and G2 - patients diagnosed at ≥18 years.
  
  
  RESULTS
  89 adults were identified: 61.8% in G1, 38.2% in G2. Gender distribution was similar in both groups. Average age in G2 was higher (38.3±8.4 vs. 26.8±6.1 years, p<0.001). Respiratory symptoms most frequently led to CF diagnosis in all patients, mainly in adulthood. There was a greater percentage of patients homozygous for the mutation delF508 in G1 (43.6 vs. 8.8%, p=0.02). Respiratory and pancreatic function, and body mass index (BMI) showed a higher severity in G1 (G1 vs. G2: FEV1: 54.6±27.3 vs. 29.9±64.6%, p=0.177; pancreatic insufficiency 72.7 vs. 26.5%, p<0.001; BMI 20.2±3.4 vs. 22.2±4.8, p=0.018). Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus were the most frequently isolated microorganisms. Lung transplantation rate was higher in G2 (20.6 vs. 10.9%, p=0.231) while mortality rate was higher in G1 (0 vs. 3.6%, p=0.261). Hospital admission rate was higher in G1 as well as mortality rate.
  
  
  CONCLUSIONS
  The results suggest that patients with CF diagnosed in childhood have characteristics that distinguish them from those diagnosed in adulthood, and these differences may have implications for diagnosis, prognosis and life expectancy.
authors.affiliation: Pulmonology Department, Centro Hospitalar de São João, EPE, Portugal. Electronic address: vferreirads@gmail.com.
authors.forename: V
authors.initials: V
authors.lastname: Santos
authors.affiliation: Pulmonology Department, Centro Hospitalar de São João, EPE, Portugal.
authors.forename: A V
authors.initials: AV
authors.lastname: Cardoso
authors.affiliation: Pulmonology Department, Centro Hospitalar de Lisboa Norte - Hospital de Santa Maria, Lisboa, Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Lopes
authors.affiliation: Pulmonology Department, Centro Hospitalar de Lisboa Norte - Hospital de Santa Maria, Lisboa, Portugal.
authors.forename: P
authors.initials: P
authors.lastname: Azevedo
authors.affiliation: Pulmonology Department, Hospitais da Universidade de Coimbra - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: F
authors.initials: F
authors.lastname: Gamboa
authors.affiliation: Pulmonology Department, Centro Hospitalar de São João, EPE, Portugal; Faculty of Medicine of Porto University, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Amorim
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies
keywords: Cystic fibrosis; Cystic fibrosis adult; Cystic fibrosis diagnosis; Cystic fibrosis in childhood; Cystic fibrosis late diagnosis