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

• Sponsor: Fondation Ophtalmologique Adolphe de Rothschild (Other)
• Overall Status: Completed
• CT.gov ID: NCT04408300
• Collaborator: (none)
• Enrollment: 124
• Location: 1
• Arm: 1
• Actual Duration (Months): 20.4
• Patients Per Site Per Month: 6.1

Study Details

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.).

Condition or Disease	Intervention/Treatment	Phase
Wilson Disease	Procedure: Ophthalmological exam	N/A

Study Design

• Study Type: Interventional
• Actual Enrollment: 124 participants
• Allocation: N/A
• Intervention Model: Single Group Assignment
• Masking: None (Open Label)
• Primary Purpose: Other
• Official Title: Study of Retinal Vascular Parameters in Patients With Wilson's Disease
• Actual Study Start Date: Jun 4, 2020
• Actual Primary Completion Date: Jan 6, 2022
• Actual Study Completion Date: Feb 15, 2022

Arms and Interventions

Arm	Intervention/Treatment
Experimental: Ophthalmological exam	Procedure: Ophthalmological exam; 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

Outcome Measures

Primary Outcome Measures

1. Measuring the width of the Kayser-Fleischer ring [Enrollment]

   OCT-B scan

2. Corrected visual acuity measurement [Enrollment]

   ETDRS

3. Intraocular pressure measurement (mmHg) [Enrollment]

4. Measurement of the thickness of the retinal nerve fibers layer (RNFL) [Enrollment]

   OCT-B

5. Measurement of the central avascular zone (in µm2) [Inclusion]

   OCT-A

6. Measurement of the capillary density of the superficial plexus (in%) [Enrollment]

   OCT-A

7. Measurement of the capillary density of the deep plexus (in%) [Enrollment]

   OCT-A

8. Measurement of photoreceptor density (per mm2) [Enrollment]

   Adaptive optics

9. Measurement of wall to lumen ratio (in %) [Enrollment]

   Adaptive optics

10. Measurement of the arterial wall section area (µm²) [Enrollment]

    Adaptive optics

11. Measurement of the diameter of the arterial lumen (µm) [Enrollment]

    Adaptive optics

Eligibility Criteria

Criteria

• Ages Eligible for Study: 18 Years and Older
• Sexes Eligible for Study: All
• Accepts Healthy Volunteers: No

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

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	Hôpital Fondation A. de Rothschild	Paris		France	75019

Sponsors and Collaborators

• Fondation Ophtalmologique Adolphe de Rothschild

Investigators

• Principal Investigator: Aurélia POUJOIS, MD, Fondation A. de Rothschild

Study Documents (Full-Text)

None provided.

More Information

Publications

None provided.

• Responsible Party: Fondation Ophtalmologique Adolphe de Rothschild
• ClinicalTrials.gov Identifier: NCT04408300
• Other Study ID Numbers: APS_2020_5
• First Posted: May 29, 2020
• Last Update Posted: Mar 3, 2022
• Last Verified: Mar 1, 2022

• Studies a U.S. FDA-regulated Drug Product: No
• Studies a U.S. FDA-regulated Device Product: No
• Keywords provided by Fondation Ophtalmologique Adolphe de Rothschild: Optical Coherence Tomography; Adaptive optics
• Additional relevant MeSH terms: Hepatolenticular Degeneration

Study Results

No Results Posted as of Mar 3, 2022