title: Cystic fibrosis in Austria.
abstract: Registry data for patients with cystic fibrosis (CF) are increasingly used to evaluate the natural history, for benchmarking of therapy and in order to identify eligible patients for clinical studies. So far, no data on frequency and clinical status of CF patients have been available for Austria on a national level. We collected data of CF patients treated 2014 in Austrian CF outpatient clinics by means of a European CF registry and on an individual search basis. A total of 773 CF patients with a median age of 18.9 years (SD 11.8 years) were seen in 13 centers (18-151 patients/center). Homozygous F508del mutation being the most common genotype was observed in 48.8% of patients. Mean age at diagnosis was 27 days. In 59% of all patients FEV1% predicted (Forced Exspiratory Volume in 1 second) was <80% and in 20% <50%. An average FEV1 predicted decline per year of 1.9% was observed between 6-18 years of age. Colonisation with Pseudomonas aeruginosa ranged between 12% and 69% in adult patients and in 0-16% in children with CF. Burkholderia cepacia complex species were present in a total of 29 samples (3.8%). Insulin therapy for diabetes was given in 14.5%. Liver involvement was reported in 36.3%. A wide variation of prescribed CF therapy was observed between centers. Data on CF patients living in Austria are now available and form a basis for clinical benchmarking as well as analyses from a public health perspective.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Wilhelminenspital, Montleartstraße 37, 1160, Vienna, Austria. thomas.frischer@wienkav.at.
authors.forename: Thomas
authors.identifier: http://orcid.org/0000-0001-5506-7884
authors.initials: T
authors.lastname: Frischer
authors.affiliation: Department of Pneumology and Allergology, University Children's Hospital of Graz, Graz, Austria.
authors.forename: Ernst
authors.initials: E
authors.lastname: Eber
authors.affiliation: Department of Pediatrics and Adolescent medicine, University Children's Hospital of Innsbruck, Innsbruck, Austria.
authors.forename: Helmut
authors.initials: H
authors.lastname: Ellemunter
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Wilhelminenspital, Montleartstraße 37, 1160, Vienna, Austria.
authors.forename: Angela
authors.initials: A
authors.lastname: Zacharasiewicz
authors.affiliation: Department of Pneumology, Krankenhaus Hietzing, Vienna, Austria.
authors.forename: Ingrid
authors.initials: I
authors.lastname: Kaluza
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Kardinal Schwarzenberg'sches Krankenhaus, Schwarzach, Austria.
authors.forename: Josef
authors.initials: J
authors.lastname: Riedler
authors.affiliation: Department of Pneumology, Allergology and Endocrinology, University Children's Hospital of Vienna, Vienna, Austria.
authors.forename: Sabine
authors.initials: S
authors.lastname: Renner
mesh_terms: D000293:Adolescent; D000328:Adult; D001317:Austria / Q000453:epidemiology; D019121:Burkholderia Infections / Q000150:complications / Q000453:epidemiology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D005838:Genotype; D006801:Humans; D007223:Infant; D008297:Male; D011552:Pseudomonas Infections / Q000150:complications / Q000453:epidemiology / Q000628:therapy; D012042:Registries; D055815:Young Adult
chemical_list: C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Cystic fibrosis; Epidemiology; Patient registry