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UK Cystic Fibrosis Registry Demographics

Population Size

11,000

People

Population Size statistic card

Years

2007 - 2025

Years statistic card

Associated BioSamples

None/not available

Associated BioSamples statistic card

Geographic coverage

UK

Geographic coverage statistic card

Lead time

2-6 months

Lead time statistic card

Summary

Cystic Fibrosis Registry Demographics record is made up of data items relating to key demographic information and clinical data on individuals with CF in the United Kingdom. The dataset includes variables such as age, sex, ethnicity, geographical location, genotype, age at diagnosis, and key clinical indicators. The dataset is structured to support epidemiological research, healthcare planning, and analysis of disparities in care and outcomes across the United Kingdom.

DOI for dataset

https://doi.org/10.1093/ije/dyx196

Documentation

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.

Dataset type

Health and disease, Treatments/Interventions, Measurements/Tests, Lifestyle, Registry

Dataset population size

11000

Associated media

https://www.cysticfibrosis.org.uk/about-us/uk-cf-registry/reporting-and-resources

Keywords

Observations

Observed Node

Disambiguating Description

Measured Value

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 Dec 2024

Provenance

Purpose of dataset collection

Disease registry, Care, Study, Research cohort, Trial, Audit

Source of data extraction

EPR, Paper-based, LIMS, Other

Collection source setting

Secondary care - Outpatients, Secondary care - In-patients, Patient report outcome, Clinic

Patient pathway description

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

Image contrast

Not stated

Biological sample availability

None/not available

Structural Metadata

Details

Publishing frequency

Annual

Version

8.0.0

Modified

08/10/2024

Distribution release date

01/01/2024

Citation Requirements

N/A

Coverage

Start date

31/10/2007

End date

31/12/2025

Time lag

2-6 months

Geographic coverage

UK

Maximum age range

120

Follow-up

Continuous

Dataset completeness

https://www.cysticfibrosis.org.uk/about-us/uk-cf-registry/reporting-and-resources

Accessibility

Language

en

Alignment with standardised data models

OTHER

Controlled vocabulary

LOCAL

Format

csv

Data Access Request

Dataset pipeline status

Available

Access rights

https://www.cysticfibrosis.org.uk/the-work-we-do/uk-cf-registry/apply-for-data-from-the-uk-cf-registry

Time to dataset access

2-6 months

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

Access method category

Varies based on project

Access service description

To be reviewed. Visit: https://www.cysticfibrosis.org.uk/about-us/uk-cf-registry

Jurisdiction

UK

Data use limitation

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

Data Controller

Cystic Fibrosis Trust

Data Processor

Cystic Fibrosis Trust

Investigations

https://doi.org/10.1016/S2213-2600(19)30385-5

Demographics

Enrichment and Linkage

Investigations

https://doi.org/10.1016/S2213-2600(19)30385-5

Dataset Types: Health and disease, Treatments/Interventions, Measurements/Tests, Lifestyle, Registry

Dataset Sub-types: Rare diseases,Metabolic and endocrine,Immunity,Others,Others,Others, Vaccines, Other diagnostics, Smoking, Disease registry (research)

Collection Sources: Secondary care - Outpatients, Secondary care - In-patients, Patient report outcome, Clinic