Version: 1.0.0 | Published: 8 Oct 2024 | Updated: 507 days ago
Summary
DOI Name:
10.1093/ije/dyx196
Documentation
Description:
UK Cystic Fibrosis Demographic Registry Dataset is a comprehensive, anonymised dataset containing demographic and clinical information on individuals diagnosed with cystic fibrosis (CF) across the United Kingdom. Maintained by the UK Cystic Fibrosis Trust, the dataset includes detailed variables such as age, sex, ethnicity, geographic region, age at diagnosis, CFTR genotype, and other clinical markers including patients genetic mutation, and chronic medication with their lung function result (FEV1).
The demographic dataset is an essential resource for researchers conducting epidemiological studies, health equity analyses, treatment efficacy assessments, and healthcare service planning. Data access is managed under strict ethical and governance frameworks, ensuring patient confidentiality. Researchers interested in accessing the dataset can find application details and supporting documentation through the UK Cystic Fibrosis Registry official website: https://www.cysticfibrosis.org.uk/the-work-we-do/uk-cf-registry. The demographic dataset enables data-driven research to support evidence-based improvements in CF care and policy development across United Kingdom.
Coverage
Spatial:
UK
Typical Age Range:
0-120
Follow Up:
Continuous
Pathway:
UK cystic fibrosis patient pathway is underpinned by a highly integrated,
multidisciplinary care model aimed at optimising health outcomes from infancy
through adulthood. The pathway adapts to the evolving needs of each patient,
guided by national standards (such as those from NICE and the UK CF Trust), and
leverages innovations such as CFTR modulators and digital health tools to
improve quality of life and longevity. Researchers from recognised institutions
can apply for access to aggregated, anonymised, or pseudonymised data from the
UK CF Registry to perform their own analysis for the benefit of people with
cystic fibrosis. The patient pathway is designed to deliver timely,
personalised, and evidence-based care at each stage of the disease. Pathway for
patient who are diagnosed with CF 1. Newborn Screening and Diagnosis: Newborn
Screening (NBS). 2. Specialist CF Centre Management 3. Ongoing Management and
Adolescence 4. Transition to Adult Care 5. Advanced Disease and End-of-Life Care
6. Data and Continuous Monitoring
Provenance
Origin
Purposes:
- Disease registry
- Care
- Study
- Research cohort
- Trial
- Audit
Sources:
- EPR
- Paper-based
- LIMS
- Other
Collection Situations:
- Secondary care - Outpatients
- Secondary care - In-patients
- Patient report outcome
- Clinic
Temporal
Accrual Periodicity:
Annual
Distribution Release Date:
01 January 2024
Start Date:
31 October 2007
End Date:
31 December 2025
Time Lag:
2-6 months
Accessibility
Access
Access Rights:
Access Service:
To be reviewed. Visit: https://www.cysticfibrosis.org.uk/about-us/uk-cf-registry
Access Request Cost:
Whilst the UK CF Registry does not charge for data, it must recoup costs of data
handling (including extract and cleaning), project management, and analysis
(where applicable) on behalf of third parties. To find out more about data
access request, visit
https://www.cysticfibrosis.org.uk/the-work-we-do/uk-cf-registry/apply-for-data-from-the-uk-cf-registry
Delivery Lead Time:
2-6 months
Jurisdictions:
UK
Data Controller:
Cystic Fibrosis Trust
Data Processor:
Cystic Fibrosis Trust
Usage
Data Use Limitations:
- Research-specific restrictions
- Not for profit use
- No linkage
Data Use Requirements:
- Ethics approval required
- Not for profit use
- Time limit on use
- Project-specific restrictions
Resource Creators:
N/A
Format and Standards
Vocabulary Encoding Schemes:
LOCAL
Conforms To:
OTHER
Languages:
en
Formats:
csv
Observations
Statistical Population
Population Description
Population Size
Measured Property
Observation Date
Findings
All observations of people with CF are recorded after their annual review encounter. The internal data dictionary is used as a scope and structure for how data should be recorded on the Registry site.
11000
Demographics Dataset
31 December 2024