Study of BEST1 Vitelliform Macular Dystrophy

NCT ID: NCT05809635

Last Updated: 2025-07-30

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

RECRUITING

Total Enrollment

52 participants

Study Classification

OBSERVATIONAL

Study Start Date

2021-03-30

Study Completion Date

2026-05-31

Brief Summary

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The purpose of this study is to establish the natural history of of participants with BESTROPHIN 1 Vitelliform Macular Dystrophy.

The blinding disorder Best Vitelliform Macular Dystrophy (VMD) is caused by any one of more than 250 different mutations in the BEST1 gene.

As new treatments are developed, a clear understanding of the natural history of disease progression of BEST1 VMD is necessary. The goals of this natural history study are to:

1. Report the natural history of retinal degeneration in participants with a clinical diagnosis of VMD with molecular confirmation of a pathogenic BEST1 mutation(s).
2. Identify sensitive structural and functional outcome measures to use for future multicenter clinical trials for the treatment of BESTROPHIN 1 VMD.
3. Compare progression of the identified structural and functional measures between the two eyes to judge the suitability of the second untreated eye as a control for a future clinical trial involving unilateral treatment
4. Identify well-defined patient populations for future clinical trials of investigative treatments for BEST1 VMD.

Detailed Description

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Conditions

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Best Vitelliform Macular Dystrophy Retinitis Pigmentosa

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Best Vitelliform Macular Dystrophy (VMD) Participants

Participants with a clinical picture of Retinitis pigmentosa with dominant and recessive variants in the BEST1 gene

Natural History Study

Intervention Type OTHER

Longitudinal assessment of participants with BEST1 Vitelliform Macular Dystrophy

Interventions

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Natural History Study

Longitudinal assessment of participants with BEST1 Vitelliform Macular Dystrophy

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Ability to provide informed consent
* Diagnosis of BEST1-associated VMD by study physician, who are trained retinal specialists in the university clinic Must be able to commit to 4 follow-up study visits (3 years)

Exclusion Criteria

* Systemic condition that prevents the participant from undergoing the exams
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Universität Tübingen

OTHER

Sponsor Role collaborator

Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts

OTHER

Sponsor Role collaborator

National Eye Institute (NEI)

NIH

Sponsor Role collaborator

Columbia University

OTHER

Sponsor Role lead

Responsible Party

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Stephen H. Tsang

Laszlo Z. Bito Professor of Ophthalmology and Professor of Pathology and Cell Biology

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Stephen H Tsang, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

Columbia University

Locations

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Columbia University Irving Medical Center

New York, New York, United States

Site Status RECRUITING

Institut de la Vision/Centre de maladies rares du Centre Hospitalier National Ophtalmologique des Quinze-Vingts

Paris, , France

Site Status NOT_YET_RECRUITING

Eberhard Karls University Tubingen

Tübingen, , Germany

Site Status RECRUITING

Countries

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United States France Germany

Central Contacts

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Stephen H Tsang, MD, PhD

Role: CONTACT

212-342-1186

Facility Contacts

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Stephen H Tsang, MD, PhD

Role: primary

212-342-1186

Isabelle Audo, MD, PhD

Role: primary

+33 1 40 02 14 30

Camille Andrieu, MD

Role: backup

+33 1 40 02 14 51

Laura Kuehlewein, MD

Role: primary

+49 07071 29-88088

Katarina Stingl, MD

Role: backup

+49 7071 29 87421

Other Identifiers

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R24EY028758

Identifier Type: NIH

Identifier Source: secondary_id

View Link

AAAT5994

Identifier Type: -

Identifier Source: org_study_id

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