title: [Improved prognosis for patients with cystic fibrosis. A result of aggressive center-based treatment].
abstract: We report survival data for Danish centre-treated cystic fibrosis (CF) patients, covering the period 1974-1993 using cross-sectional cumulative survival probability based on annual age-specific mortality rates. No significant differences were noted in the survival probability when patients were grouped according to sex or absence/presence of meconium ileus. The annual mortality rate for 1989-1993 was 0-1.2%. Using the age-specific mortality rate for 1989-1993, we were unable to calculate the median survival probability because the curve did not fall below 50% (age up to 45 years). It was, however, possible to show that the survival probability for a CF child born after 1989 to reach his or hers 45th birthday was 80.4% (95% confidence interval 76.5-84.6%). The probability of surviving 40 years after the diagnosis of CF is made was 83.3% (95% confidence interval 80.1%-86.6%). This is considerably higher than any other published survival probability. An aggressive anti-Pseudomonas aeruginosa treatment regimen seemed important in achieving the observed improved survival.
authors.affiliation: Rigshospitalet, cystisk fibrose center afsnit 5003, og klinisk mikrobiologisk afdeling.
authors.forename: B
authors.initials: B
authors.lastname: Frederiksen
authors.forename: S L
authors.initials: SL
authors.lastname: Thorsteinsson
authors.forename: C
authors.initials: C
authors.lastname: Koch
authors.forename: N
authors.initials: N
authors.lastname: Høiby
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000382:microbiology / Q000401:mortality* / Q000628:therapy; D003718:Denmark; D005260:Female; D006801:Humans; D008297:Male; D018802:Patient-Centered Care; D018410:Pneumonia, Bacterial / Q000188:drug therapy / Q000382:microbiology / Q000517:prevention & control; D011379:Prognosis; D011552:Pseudomonas Infections / Q000188:drug therapy / Q000517:prevention & control; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification