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Cystic Fibrosis Patient Annual Review Encounters

Population Size

11,000

People

Population Size statistic card

Years

1996 - 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

The Cystic Fibrosis Annual Review Record dataset contains structured clinical information collected during routine yearly assessments of individuals with cystic fibrosis. It includes data on lung function, nutritional status, microbiology, medications, complications, and other clinical indicators. The dataset captures longitudinal health information across multiple years, supporting the analysis of disease progression and treatment outcomes.

DOI for dataset

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

Documentation

The Cystic Fibrosis Annual Review Dataset contains routinely collected clinical data from annual review assessments of individuals diagnosed with cystic fibrosis. These reviews are part of standard clinical care and include comprehensive evaluations of respiratory health, nutritional status, treatment history, and disease complications. The annual review dataset supports research into disease progression, treatment effectiveness, and clinical outcomes in cystic fibrosis. It can be used for epidemiological studies, healthcare planning, and evaluation of new interventions. For questions or data access requests, please contact: www.cysticfibrosis.org.uk/registry Data Resource Profile: The UK CF Registry' published in the International Journal of Epidemiology (2018 Feb 1;47(1)9-10e). https://www.cysticfibrosis.org.uk/news/key-findings-from-the-2023-uk-cf-registry-report https://www.cysticfibrosis.org.uk/about-us/uk-cf-registry/reporting-and-resources

Dataset type

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

Dataset population size

11000

Associated media

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

Synthetic data web link

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 are used as a scope and structure for how data should be recorded on the UKCF registry site.

11000

The UKCF annual review record is data entry cut-off is 31st of January every year. People with CF have a total count of AR entry is calculated each year and on average is about 36,000

01 Mar 2015

Provenance

Purpose of dataset collection

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

Source of data extraction

EPR, Paper-based, LIMS

Collection source setting

Clinic, Secondary care - Outpatients, Prescribing - Hospital, Patient report outcome, Secondary care - In-patients, Community, Home

Patient pathway description

The dataset contains: time-invariant variables, such as sex, genotype and date of birth and longitudinal variables that change over time, such as weight and measures of lung function. CF patients are seen in the outpatient clinic for a comprehensive annual review, including evaluation of clinical status, pulmonary function, microbiology of respiratory tract secretions and use of major CF-related therapies. The minimum data collection requirement for the UK CF Registry is an annual dataset, usually taken from the annual review clinic visit. The data collected at the annual review are indicated in the dataset, and can be distinguished from &ampampampampampampampampampampampampampampampampampampampamplsquoencounter&ampampampampampampampampampampampampampampampampampampampamprsquo-based data collected in the interval between clinic visits. The key areas where data are collected during an annual review includes: Core information such as weight, height and BMi during annual review, Hospital admissions and intravenous therapies, Pulmonary function, Chronic medications, Culture and microbiology, Health complications, Nutritional assessment, Physiotherapy,

Lifestyle and outcomes (death and transplants). The annual review process is designed to capture a comprehensive snapshot of a patient&ampampampampampampampampampampampampampampampampampampampamp#039s health status

, facilitating the monitoring of disease progression and the effectiveness of treatments.

Image contrast

Not stated

Biological sample availability

None/not available

Structural Metadata

Details

Publishing frequency

Annual

Version

7.0.0

Modified

08/10/2024

Distribution release date

31/08/2019

Citation Requirements

N/A

Coverage

Start date

01/01/1996

End date

29/05/2025

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/2024-registry-report-highlights

Accessibility

Language

en

Alignment with standardised data models

OTHER, NHS DATA DICTIONARY, NHS SCOTLAND DATA DICTIONARY, NHS WALES DATA DICTIONARY, LOINC

Controlled vocabulary

LOCAL, NHS NATIONAL CODES, NHS SCOTLAND NATIONAL CODES, NHS WALES NATIONAL CODES, ODS

Format

csv, xlsl

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. The charging structure outlined below is designed to be fair, proportionate, and transparent whilst furthering the Registry aim of stimulating research use of the data for the benefit of people with cystic fibrosis. 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

Researchers from recognised institutions can apply for access to aggregated, anonymised, or pseudonymised data from the UK CF Registry. The application process involves completing a Data Request form and submitting it to the Cystic Fibrosis Trust. The Registry Research Committee reviews applications, with decisions typically provided within 1-3 months of request submission. Data requests generally take a minimum of eight-to twelve weeks to complete. While the UK CF Registry does not charge for data access, it recoups costs associated with data handling, including extraction, cleaning, project management, and analysis. This charging structure is designed to be fair, proportionate, and transparent, aiming to stimulate research use of the data for the benefit of people with cystic fibrosis.

Jurisdiction

GB

Data use limitation

Research-specific restrictions,Not for profit use,No linkage,Project-specific restrictions

Data use requirements

Ethics approval required,Project-specific restrictions,Collaboration required,Not for profit use,Time limit on use,Disclosure control,User-specific restriction

Data Controller

Cystic Fibrosis Trust Dr Sarah Clarke is the Associate Director of Data and Quality Improvement Elaine Gunn is the Registry Clinical Data Manager

Data Processor

Cystic Fibrosis Trust NHS England NHS Wales NHS Scotland NHS Northern Ireland

Investigations

https://www.cysticfibrosis.org.uk/get-involved/clinical-trials/trialstracker/tt005796, https://cfstart.org.uk/

Demographics

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

Dataset Sub-types: Rare diseases,Others,Others, Vaccines, Other diagnostics, Marital status,Ethnicity, Smoking, Disease registry (research)

Collection Sources: Clinic, Secondary care - Outpatients, Prescribing - Hospital, Patient report outcome, Secondary care - In-patients, Community, Home

Relationships:

Publications about this dataset

Whole-exome sequencing reveals a role of HTRA1 and EGFL8 in brain white matter hyperintensities.Malik R, Beaufort N, Frerich S, Gesierich B, Georg...

Brain : a journal of neurology

Published - 2021