Global Registry for COL6-related Dystrophies

NCT ID: NCT04020159

Last Updated: 2023-03-22

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

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Recruitment Status

UNKNOWN

Total Enrollment

1000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-08-01

Study Completion Date

2024-10-31

Brief Summary

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The Global Registry for COL6-related dystrophies (www.collagen6.org) is a database for individuals who have been diagnosed with Bethlem Myopathy, Ullrich Congenital Muscular Dystrophy (UCMD) or an intermediate form of these diseases. The registry team is based at the John Walton Muscular Dystrophy Research Centre at Newcastle University, UK and is part of the TREAT-NMD alliance global network of registries. The registry has been developed in partnership with a number of leading neuromuscular researchers and is funded by the Collagen VI Alliance.

This patient registry will:

* Help identify patients for relevant clinical trials as they become available
* Encourage further research into Collagen 6-related dystrophies
* Provide researchers with specific patient information to support their research
* Assist doctors and other health professionals by providing them with up-to-date information on managing Collagen 6- related dystrophies, to help them deliver better standards of care for their patients

The investigators welcome the registration of:

✓ All patients, with a diagnosis of a COL6-related dystrophy (Bethlem Myopathy, Ullrich Congenital Muscular Dystrophy or Intermediate form) , which has been confirmed via genetic testing or muscle biopsy.

Detailed Description

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The Global Registry for COL6-related dystrophies (www.collagen6.org) is an international registry for patients with a COL6-related condition; no experimental intervention is involved. Patients will receive information on the most up to date standards of care relating to their disease and may be invited to participate in relevant clinical trials. Their data will be updated annually and stored indefinitely, or until they request their data to be removed.

The data will be collected via a secure online form and stored on a secure server. Data collected from patients will include demographic information, diagnosis, current condition (wheelchair use and motor ability, respiratory function, contractures and scoliosis), family history and quality of life. Further information collected from patients' doctors will include genetics, age of onset, lung function, medication, muscle strength, contractures and muscle MRI findings.

One of the purposes of the Global Registry for COL6-related dystrophies is to define the patient population and disease course, therefore data is collected longitudinally and participants will be invited to update their records on a yearly basis.

Access to the database is limited to named individuals, approved by the Principal Investigator, Professor Volker Straub, and the database curator. The database is password protected, with different levels of access. All database entries and contacts are traceable through an audit trail. A SOP has been formulated to cover administration, management and communication procedures for the database.

Researchers and Industry may ask specific questions of registry data. These questions may be to support academic research, feasibility studies for clinical trials or recruitment for clinical trials. Any enquiries for data will be examined and approved by the registry steering committee. Data released for approved enquiries would be de-identified aggregate data. In the case of recruitment for clinical trials when the registry principle investigator and/or curator believe that a patient meets a trial's inclusion criterion and might benefit from participation in a trial, the patient will be contacted by the curator with general information about the upcoming trial and will be asked to contact their local trial/study centre for more information if they are interested in participating. The patient's name or any other personal information will not be given to researchers or industry. Clinical trials will need to have appropriate IRB/ethics board and steering committee approval although the registry will not endorse any particular trial.

Registry participation in voluntary and participants may decline to participate or withdraw consent for their data to be stored in the Global Registry for COL6-related dystrophies at any time without prejudice.

The protocol, patient information sheets and consent forms, and relevant supporting information has been reviewed and approved by NHS HRA Research Ethics Committee and Newcastle University ethics committee.

Conditions

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Bethlem Myopathy Ullrich Congenital Muscular Dystrophy 1, Digenic, Col6A1/Col6A2 Ullrich Congenital Muscular Dystrophy 1, Autosomal Recessive Ullrich Congenital Muscular Dystrophy 1, Autosomal Dominant Bethlem Myopathy 1, Autosomal Recessive UCMD BTHLM1

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Participants with COL6-related dystrophy

Participants who have volunteered to participate will complete various questionnaires relating to their condition.

Patient Registry

Intervention Type OTHER

No intervention/treatment

Interventions

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Patient Registry

No intervention/treatment

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* confirmed diagnosis of a COL6-related dystrophy (Bethlem Myopathy, Ullrich Congenital Muscular Dystrophy (UCMD), or an intermediate form of these conditions.

Exclusion Criteria

* absence of a diagnosis of COL6-related dystrophy
* Bethlem Myopathy Type 2, Ullrich Congenital Muscular Dystrophy 2 and other COL12-related conditions
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Newcastle-upon-Tyne Hospitals NHS Trust

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Volker Straub

Role: PRINCIPAL_INVESTIGATOR

Newcastle University

Locations

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Newcastle University

Newcastle upon Tyne, , United Kingdom

Site Status RECRUITING

Countries

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United Kingdom

Central Contacts

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Sam McDonald

Role: CONTACT

01912418605

Sam McDonald

Role: CONTACT

Facility Contacts

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Sam McDonald

Role: primary

0191 2418605

Other Identifiers

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8451

Identifier Type: OTHER

Identifier Source: secondary_id

18/NE/0243

Identifier Type: -

Identifier Source: org_study_id

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