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Study of Retinal Vascular Parameters in Patients With Wilson's Disease (WILLY)

Primary Purpose

Wilson Disease

Status
Completed
Phase
Not Applicable
Locations
France
Study Type
Interventional
Intervention
Ophthalmological exam
Sponsored by
Fondation Ophtalmologique Adolphe de Rothschild
About
Eligibility
Locations
Arms
Outcomes
Full info

About this trial

This is an interventional other trial for Wilson Disease focused on measuring Optical Coherence Tomography, Adaptive optics

Eligibility Criteria

18 Years - undefined (Adult, Older Adult)All SexesDoes not accept healthy volunteers

Inclusion Criteria:

  • Wilson's disease with Ferenci score ≥4

Exclusion Criteria:

  • Neurological or behavioral handicap that does not allow for ophthalmological examinations
  • Hepatic decompensation that does not allow for ophthalmological examinations
  • Pregnant or breastfeeding woman

Sites / Locations

  • Hôpital Fondation A. de Rothschild

Arms of the Study

Arm 1

Arm Type

Experimental

Arm Label

Ophthalmological exam

Arm Description

Outcomes

Primary Outcome Measures

Measuring the width of the Kayser-Fleischer ring
OCT-B scan
Corrected visual acuity measurement
ETDRS
Intraocular pressure measurement (mmHg)
Measurement of the thickness of the retinal nerve fibers layer (RNFL)
OCT-B
Measurement of the central avascular zone (in µm2)
OCT-A
Measurement of the capillary density of the superficial plexus (in%)
OCT-A
Measurement of the capillary density of the deep plexus (in%)
OCT-A
Measurement of photoreceptor density (per mm2)
Adaptive optics
Measurement of wall to lumen ratio (in %)
Adaptive optics
Measurement of the arterial wall section area (µm²)
Adaptive optics
Measurement of the diameter of the arterial lumen (µm)
Adaptive optics

Secondary Outcome Measures

Full Information

First Posted
May 26, 2020
Last Updated
March 2, 2022
Sponsor
Fondation Ophtalmologique Adolphe de Rothschild
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1. Study Identification

Unique Protocol Identification Number
NCT04408300
Brief Title
Study of Retinal Vascular Parameters in Patients With Wilson's Disease
Acronym
WILLY
Official Title
Study of Retinal Vascular Parameters in Patients With Wilson's Disease
Study Type
Interventional

2. Study Status

Record Verification Date
March 2022
Overall Recruitment Status
Completed
Study Start Date
June 4, 2020 (Actual)
Primary Completion Date
January 6, 2022 (Actual)
Study Completion Date
February 15, 2022 (Actual)

3. Sponsor/Collaborators

Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Fondation Ophtalmologique Adolphe de Rothschild

4. Oversight

Studies a U.S. FDA-regulated Drug Product
No
Studies a U.S. FDA-regulated Device Product
No

5. Study Description

Brief Summary
The rare disease reference center " Wilson disease and other rare copper-related diseases" of the Rothschild Foundation follows a large number of patients with Wilson's with varying degrees of impairment and located at different times of their care. Many people with Wilson's disease have a characteristic greenish-brown ring, known as Kayser-Fleischer, appearing at the periphery of the cornea due to a deposit of copper at the Descemet membrane. As a general rule, if the patient is compliant with his treatment, the ring usually disappears within a few years, although it may persist in some patients. However, apart from the stage of diagnosis, and the evolution of the ring, ophthalmological examinations are little used for the follow-up of these patients. The objective of this study is to describe the retinal parameters, in particular vascular with two new retinal imaging technologies (OCT-A :Optical Coherence Tomography-Angiography , Adaptive optics) in patients with Wilson's disease and to correlate them with the parameters of the usual follow-up of these patients (hepatic assessment, exchangeable plasma copper, neurological scores, compliance, etc.).

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Wilson Disease
Keywords
Optical Coherence Tomography, Adaptive optics

7. Study Design

Primary Purpose
Other
Study Phase
Not Applicable
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
N/A
Enrollment
124 (Actual)

8. Arms, Groups, and Interventions

Arm Title
Ophthalmological exam
Arm Type
Experimental
Intervention Type
Procedure
Intervention Name(s)
Ophthalmological exam
Intervention Description
Retinophotography, OCT-A (Optical Coherence Tomography- Angiography), OCT-B scan (Optical Coherence Tomography B Scan), Measurement of corrected visual acuity in ETDRS (Early Treatment Diabetic Retinopathy Study), Measurement of intraocular pressure, adaptive optics
Primary Outcome Measure Information:
Title
Measuring the width of the Kayser-Fleischer ring
Description
OCT-B scan
Time Frame
Enrollment
Title
Corrected visual acuity measurement
Description
ETDRS
Time Frame
Enrollment
Title
Intraocular pressure measurement (mmHg)
Time Frame
Enrollment
Title
Measurement of the thickness of the retinal nerve fibers layer (RNFL)
Description
OCT-B
Time Frame
Enrollment
Title
Measurement of the central avascular zone (in µm2)
Description
OCT-A
Time Frame
Inclusion
Title
Measurement of the capillary density of the superficial plexus (in%)
Description
OCT-A
Time Frame
Enrollment
Title
Measurement of the capillary density of the deep plexus (in%)
Description
OCT-A
Time Frame
Enrollment
Title
Measurement of photoreceptor density (per mm2)
Description
Adaptive optics
Time Frame
Enrollment
Title
Measurement of wall to lumen ratio (in %)
Description
Adaptive optics
Time Frame
Enrollment
Title
Measurement of the arterial wall section area (µm²)
Description
Adaptive optics
Time Frame
Enrollment
Title
Measurement of the diameter of the arterial lumen (µm)
Description
Adaptive optics
Time Frame
Enrollment

10. Eligibility

Sex
All
Minimum Age & Unit of Time
18 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
Inclusion Criteria: Wilson's disease with Ferenci score ≥4 Exclusion Criteria: Neurological or behavioral handicap that does not allow for ophthalmological examinations Hepatic decompensation that does not allow for ophthalmological examinations Pregnant or breastfeeding woman
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Aurélia POUJOIS, MD
Organizational Affiliation
Fondation A. de Rothschild
Official's Role
Principal Investigator
Facility Information:
Facility Name
Hôpital Fondation A. de Rothschild
City
Paris
ZIP/Postal Code
75019
Country
France

12. IPD Sharing Statement

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Study of Retinal Vascular Parameters in Patients With Wilson's Disease

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