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.
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 UKPPpatients (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.
Authors: Jenna Sykes; Sanja Stanojevic; Christopher H Goss; Bradley S Quon; Bruce C Marshall; Kristofer Petren; Josh Ostrenga; Aliza Fink; Alexander Elbert; Anne L Stephenson Journal: J Clin Epidemiol Date: 2015-10-03 Impact factor: 6.437
Authors: Rémy Choquet; Meriem Maaroufi; Albane de Carrara; Claude Messiaen; Emmanuel Luigi; Paul Landais Journal: J Am Med Inform Assoc Date: 2014-07-18 Impact factor: 4.497
Authors: Anne L Stephenson; Jenna Sykes; Sanja Stanojevic; Bradley S Quon; Bruce C Marshall; Kristofer Petren; Josh Ostrenga; Aliza K Fink; Alexander Elbert; Christopher H Goss Journal: Ann Intern Med Date: 2017-03-14 Impact factor: 25.391
Authors: Stephen W Turner; Jon G Ayres; Tatiana V Macfarlane; Anil Mehta; Gita Mehta; Colin N Palmer; Steve Cunningham; Tim Adams; Krishnan Aniruddhan; Claire Bell; Donna Corrigan; Jason Cunningham; Andrew Duncan; Gerard Hunt; Richard Leece; Una MacFadyen; Jonathan McCormick; Sally McLeish; Andrew Mitra; Deborah Miller; Elizabeth Waxman; Alan Webb; Slawomir Wojcik; Somnath Mukhopadhyay; Donald Macgregor Journal: BMC Med Res Methodol Date: 2010-12-06 Impact factor: 4.615