Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia.
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).


CONCLUSIONS
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.
McCormick
Jonathan
J
United 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
Sims
Erika J
EJ
Green
Michael W
MW
Mehta
Gita
G
Culross
Frank
F
Mehta
Anil
A
D000293:Adolescent; D001315:Australia; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis; D019992:Databases as Topic; D005602:France; D006801:Humans; D007223:Infant; D012042:Registries; D006113:United Kingdom; D014481:United States