Cohort Research on Wilson's Disease

NCT ID: NCT04212195

Last Updated: 2019-12-26

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

UNKNOWN

Total Enrollment

500 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-12-06

Study Completion Date

2021-12-06

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

Wilson's disease (WD) is an inherited disorder that causes abnormal copper accumulation in the brain and/or liver. Some people develop neurological or psychiatric symptoms whereas other develop liver disease. The reasons for this are unclear but genetic factors are likely to contribute. Current treatment, using copper-binding medications, is required lifelong. Some respond well but others suffer debilitating side-effects or deteriorate despite treatment, leading to disability or the need for liver transplantation.

In the first part of this study the main aim is to identify genetic factors that determine whether someone with a diagnosis of WD will develop neurological involvement or not. The investigators will invite 500 adults with WD across the UK to take part. Participants will be asked to complete an online questionnaire and provide a saliva sample for genetic testing using a collection kit sent via post. Identifying these genetic factors would significantly advance our understanding of the disease and may provide new targets for drug discovery or help guide more personalised approaches to treatment.

In the second part of this study the main aim is to develop new ways to monitor the effect of WD on the brain using tests. Copper levels in blood and urine, currently used to monitor the disease, are unreliable and do not necessarily reflect ongoing brain damage. The role of MRI scans, cerebrospinal fluid tests or other measures of brain damage, commonly used in other neurological disorders, is unclear. The investigators will therefore follow a group of 40 patients using clinical assessments and a combination of neurological tests, including novel imaging and laboratory techniques, over 24 months. Developing new approaches to monitoring the effect of WD on the brain will enable better prevention of neurological disability and be essential for demonstrating the effectiveness of new treatments, such as gene therapy, in clinical trials in the future.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Wilson's Disease

Keywords

Explore important study keywords that can help with search, categorization, and topic discovery.

Genetic modifiers Biomarkers

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Part 1

Genetic determinants (n=500)

Next generation sequencing

Intervention Type GENETIC

Saliva samples

Part 2

Biomarker discovery (n=40)

Imaging and fluid biomarkers

Intervention Type DIAGNOSTIC_TEST

Magnetic resonance imaging of the brain and urine, blood and cerebrospinal fluid sampling

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Next generation sequencing

Saliva samples

Intervention Type GENETIC

Imaging and fluid biomarkers

Magnetic resonance imaging of the brain and urine, blood and cerebrospinal fluid sampling

Intervention Type DIAGNOSTIC_TEST

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

* Diagnosed with Wilson's disease
* Age 16 years or over
* Living in the UK

Exclusion Criteria

* Participant has another medical or psychiatric illness that would interfere in completing assessments
* Participant is pregnant
Minimum Eligible Age

16 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

University College, London

OTHER

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Thomas Warner

Role: PRINCIPAL_INVESTIGATOR

UCL Queen Square Institute of Neurology

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Birmingham Women's and Children's NHS Foundation Trust

Birmingham, , United Kingdom

Site Status RECRUITING

University Hospitals Birmingham NHS Foundation Trust

Birmingham, , United Kingdom

Site Status RECRUITING

Cambridge University Hospitals NHS Foundation Trust

Cambridge, , United Kingdom

Site Status RECRUITING

Cardiff and Vale University Health Board

Cardiff, , United Kingdom

Site Status RECRUITING

King's College Hospital NHS Foundation Trust

London, , United Kingdom

Site Status RECRUITING

National Hospital for Neurology and Neurosurgery

London, , United Kingdom

Site Status RECRUITING

Royal Free London NHS Foundation Trust

London, , United Kingdom

Site Status RECRUITING

Manchester University NHS Foundation Trust

Manchester, , United Kingdom

Site Status RECRUITING

Newcastle upon Tyne Hospitals NHS Foundation Trust

Newcastle, , United Kingdom

Site Status RECRUITING

Salford Royal NHS Foundation Trust

Salford, , United Kingdom

Site Status RECRUITING

Sheffield Teaching Hospitals NHS Foundation Trust

Sheffield, , United Kingdom

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

United Kingdom

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Samuel Shribman

Role: CONTACT

Phone: 02076794025

Email: [email protected]

Facility Contacts

Find local site contact details for specific facilities participating in the trial.

David Nicholl

Role: primary

William Griffiths

Role: primary

Kathryn Peall

Role: primary

Adrian Bomford

Role: primary

Thomas Warner

Role: primary

Emmanouil Tsochatzis

Role: primary

Harpreet Dhaliwal

Role: primary

Steve Masson

Role: primary

John Ealing

Role: primary

Oliver Bandmann

Role: primary

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

18/0200

Identifier Type: -

Identifier Source: org_study_id