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Apples to apples? Comparative analyses of national CF registries
  1. Lucy Mary Perrem1,
  2. Paul McNally2,3,
  3. Felix Ratjen4
  1. 1Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada
  2. 2CF Research Group, National Children's Research Centre, Dublin, Ireland
  3. 3Department of Paediatrics, Royal College of Surgeons in Ireland, Dublin, Ireland
  4. 4Department of Pediatrics; Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada
  1. Correspondence to Professor Felix Ratjen, Department of Pediatrics; Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, M5G 1X8, Canada; felix.ratjen{at}sickkids.ca

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National registries play a crucial role in understanding the natural history of cystic fibrosis (CF) and the impact of care delivery and interventions on outcomes. Data on more than 90 000 individuals are captured in CF registries around the world.1 Detailed examination of clinical characteristics, interventions and outcomes, benchmarking CF centres within jurisdictions and international comparisons across healthcare systems can stimulate quality improvement initiatives and drive effective changes in practice.

In a 2015 cross-sectional comparison of the US and the UK national CF registries, children in the US had better lung function than their UK counterparts2; these differences persisted into early adulthood. However, both cohort effects and survivor bias could have affected the data interpretation as outlined in an accompanying editorial.3 In this issue of the Journal, addressing some of these confounders, Schlüter and colleagues4 once again compared US and UK registry data, but this time focused on longitudinal trajectories of parallel cohorts limiting the study population to children between 6 and 18 years and those homozygous for the F508del mutation. The mean difference in per cent-predicted FEV1 (ppFEV1) between the US and UK populations at ages 6, 12 and 17 years was approximately 4%, 5% and 6%, respectively. The data suggested that children in the US may also have a slower rate of lung function decline, but the evidence for this was less convincing and dependent on …

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Footnotes

  • Twitter @luperr84

  • Contributors All authors conceptualised the manuscript. LMP wrote the first draft that was reviewed and edited by PMN and FR.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.

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