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

Not Applicable

Completed

• Conditions: Wilson Disease
• Interventions: Procedure: Ophthalmological exam

• Registration Number: NCT04408300
• Lead Sponsor: Fondation Ophtalmologique Adolphe de Rothschild

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.).

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2020-05-26
• Study First Submitted QC: 2020-05-26
• Study First Posted: 2020-05-29
• Study Start: 2020-06-04
• Primary Completion: 2022-01-06
• Study Completion: 2022-02-15
• Status Verified: 2022-03
• Last Update Submitted: 2022-03-02
• Last Update Posted: 2022-03-03

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 124

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Ophthalmological exam	Ophthalmological exam	-

Primary Outcome Measures

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

Trial Locations

Locations (1)

Hôpital Fondation A. de Rothschild; 🇫🇷 Paris, France