title: [Cystic fibrosis in adults: experience of a national reference center].
abstract: BACKGROUND
  Although in most patients the diagnosis of cystic fibrosis (CF) is made during their first years of life, a proportion of patients is diagnosed during adulthood.
  
  
  OBJECTIVE
  To describe the features of adult patients diagnosed with CF.
  
  
  METHODS
  Analysis of medical records of patients diagnosed with CF in a public hospital devoted to respiratory diseases. Demographic, clinical, laboratory and microbiological data were recorded.
  
  
  RESULTS
  Fifty eight patients aged 25.4 ± 6.5 years were included. In 40% of them, CF was diagnosed after 15 years of age. The most common mutation found was AF508. Among clinical characteristics, lung involvement, mainly bronchiectasis, was found in 93%. The mean forced expiratory volume in the first second (FEV,) was 65,7 ± 27,1%. Fifteen patients were colonized with Pseudomonas aeruginosa. The main complication seen was hemoptysis, in 12% of patients. Five patients died, mostly due to respiratory distress associated with sepsis, while three were subjected to bilateral lung transplantation. Patients in whom the diagnosis of CF was made after 15 years of age, had lower frequency of AF508 mutation, were most commonly women and had a lower rate of pancreatic involvement.
  
  
  CONCLUSIONS
  CF is a disease that is increasingly reaching adult population. CF must be suspected in adolescents and young adults who suffer chronic lung diseases such as bronchiectasis, particularly when they are colonized by Pseudomonas aeruginosa, or in patients who develop infections by uncommon organisms.
authors.affiliation: Instituto Nacional del Tórax, Santiago, Chile. pafeva99@hotmail.com
authors.forename: Patricia
authors.initials: P
authors.lastname: Fernández
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Labarca
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002677:Chile; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000235:genetics / Q000382:microbiology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D009154:Mutation; D012140:Respiratory Tract Diseases / Q000150:complications; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator