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

Wilson Disease Clinical Trial

— WILLY  

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04408300
• Other study ID #: APS_2020_5
• Status: Completed
• Phase: N/A
• Start date: June 4, 2020
• Est. completion date: February 15, 2022

Study information

• Verified date: March 2022
• Source: Fondation Ophtalmologique Adolphe de Rothschild
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.).

Recruitment information / eligibility

• Status: Completed
• Enrollment: 124
• Est. completion date: February 15, 2022
• Est. primary completion date: January 6, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Wilson Disease

Intervention

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

Locations

Country	Name	City	State
France	Hôpital Fondation A. de Rothschild	Paris

Sponsors (1)

Lead Sponsor	Collaborator
Fondation Ophtalmologique Adolphe de Rothschild

Country where clinical trial is conducted

France, 

Outcome

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