Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia
Jonathan McCormick1, Erika J Sims, Michael W Green
1United Kingdom Cystic Fibrosis Database, Tayside Institute of Child Health, Ninewells Hospital and Medical School, University of Dundee, Dundee, Scotland, UK DD1 9SY. j.d.mccormick@dundee.ac.uk
Insights
This study compared UK paediatric cystic fibrosis (CF) patients to international registries. UK children with CF were older at last follow-up but had similar lung function to French patients.
Area of Science:
- Pediatric pulmonology
- Epidemiology
- Public health
Background:
- Analysis of the UK Cystic Fibrosis Database (UKCFD) for the UK paediatric population (UKPP).
- Comparison of UKPP health status (biographical, clinical, infection) with US, French, and Australasian CF Registries.
Purpose of the Study:
- To compare the health and outcomes of the UK paediatric CF population with international CF registries.
- To identify areas for improved data collection and international standardization.
Main Methods:
- Utilized UKCFD data for 2,673 patients under 18 years old in 2001.
- Compared UKPP data with the most recent available reports from other national CF registries.
Main Results:
- UKPP had the oldest median age (15.0 years); Australasian population had the youngest median age at diagnosis (1.8 months).
- UKPP showed higher proportions below the 10th centile for height (23%) and weight (19%) compared to expected, similar to Australasia.
- UKPP and French populations had comparable rates of FEV1 >80% predicted (53% vs. 54%).
Conclusions:
- Independent development of national CF registry data systems is a first step towards international comparisons.
- Standardization of data collection criteria and definitions for national CF registries is necessary.
- A standardized minimum data set is proposed to facilitate global CF registry data integration.
Background:
Using the UK Cystic Fibrosis Database, we analysed the health of the UK CF paediatric population (UKPP) in terms of their biographical, clinical and infection status and compared outcomes with the US, French and Australasian CF Registries.
Methods:
UKPP data were collected for 2,673 patients aged less than 18 years in 2001 and used as a reference base for comparison with the most recent equivalent CF Registry reports.
Results:
Although differences exist between National CF Registries, all record similar demographic factors and key outcomes. Where plausible comparisons can be made, we report that the UKPP had the oldest median age (15.0 years), the Australasian population had the lowest median age at diagnosis (1.8 months). Approximately, double the expected number of UKPP patients (23% and 19%, respectively) fall below the 10th centile for height and weight with similar outcomes in Australasia. UKPP and French populations had similar proportions with FEV1 >80% predicted (53% and 54%, respectively).
Conclusion:
Each Registry's data systems have developed independently providing a first step towards international comparisons. Standardisation of data collection criteria and definition for national CF Registries is required and we propose a standardised minimum data set, which would facilitate data integration as part of a global Registry for CF.
