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

Overview

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.).

Full Title of Study: “Study of Retinal Vascular Parameters in Patients With Wilson’s Disease”

Study Type

  • Study Type: Interventional
  • Study Design
    • Allocation: N/A
    • Intervention Model: Single Group Assignment
    • Primary Purpose: Other
    • Masking: None (Open Label)
  • Study Primary Completion Date: June 2022

Interventions

  • 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

Arms, Groups and Cohorts

  • Experimental: Ophthalmological exam

Clinical Trial Outcome Measures

Primary Measures

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

Participating in This Clinical Trial

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
  • Gender Eligibility: All

    Minimum Age: 18 Years

    Maximum Age: N/A

    Are Healthy Volunteers Accepted: No

    Investigator Details

    • Lead Sponsor
      • Fondation Ophtalmologique Adolphe de Rothschild
    • Provider of Information About this Clinical Study
      • Sponsor
    • Overall Official(s)
      • Aurélia POUJOIS, MD, Principal Investigator, Fondation A. de Rothschild
    • Overall Contact(s)
      • Amélie YAVCHITZ, MD, (0)148036454, ayavchitz@for.paris

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