title: Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening.
abstract: OBJECTIVE
  National data registries for cystic fibrosis (CF) enable comparison of health statistics between countries. We examined the US and Australian CF data registries to compare demographics, clinical practice and outcome measures.
  
  
  METHODS
  We compared the 2003 US and Australian registries. Differences in pulmonary and growth outcomes were assessed by creating models controlling for differences in age, gender, genotype, and diagnosis after newborn screening.
  
  
  RESULTS
  Data on 12 994 US and 1220 Australian patients aged ≤18 years were analyzed. A significant difference was noted in the proportion who had been diagnosed after newborn screening (Australian 65.8% vs United States 7.2%; P < .001). Australian children had significantly greater mean height percentile (41.0 vs 32.6; P < .001) and weight percentile (43.5 vs 36.1; P = .028) than US children. Mean forced expiratory volume in 1 second (FEV(1)) percent predicted adjusted for age, gender, and genotype was similar in the 2 countries (P = .80). Patients diagnosed after newborn screening had higher mean FEV(1) (5.3 [95% confidence interval (CI): 3.6-7.0]) percent predicted and BMI (0.26 [95% CI: 0.09-0.43]). Mean FEV(1) of Australian patients diagnosed after newborn screening was lower by 5.2 (95% CI: 2.8-7.6) percent predicted compared with US children.
  
  
  CONCLUSIONS
  Children diagnosed with CF after newborn screening benefited from better lung function and BMI than those diagnosed clinically. The benefit of newborn screening on lung function was significantly less in Australian children compared with US children. Statistical comparisons between CF registries are feasible and can contribute to benchmarking and improvements in care.
authors.affiliation: Department of Respiratory Medicine, Sydney Children’s Hospital,Randwick, New South Wales, Australia.
authors.forename: Bradley
authors.initials: B
authors.lastname: Martin
authors.forename: Michael S
authors.initials: MS
authors.lastname: Schechter
authors.forename: Adam
authors.initials: A
authors.lastname: Jaffe
authors.forename: Peter
authors.initials: P
authors.lastname: Cooper
authors.forename: Scott C
authors.initials: SC
authors.lastname: Bell
authors.forename: Sarath
authors.initials: S
authors.lastname: Ranganathan
mesh_terms: D000367:Age Factors; D001315:Australia; D019985:Benchmarking / Q000592:standards; D015992:Body Mass Index; D003431:Cross-Cultural Comparison; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000453:epidemiology* / Q000235:genetics; D005240:Feasibility Studies; D005260:Female; D005541:Forced Expiratory Volume; D005838:Genotype; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening / Q000592:standards; D017063:Outcome Assessment, Health Care; D058996:Quality Improvement / Q000592:standards; D012042:Registries; D012737:Sex Factors; D014481:United States