Study of Retinal Vascular Parameters in Patients With Wilson's Disease
Trial Snapshot
- Phase
- Not Applicable
- Status
- Completed
- Enrollment
- 124
- Locations
- 2
- Primary Endpoint
- Measurement of the diameter of the arterial lumen (µm)
Study Overview
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.).
Study Design
- Study Type
- Interventional
- Allocation
- Na
- Intervention Model
- Single Group
- Primary Purpose
- Other
- Masking
- None
Eligibility Criteria
- Ages
- 18 Years to — (Adult, Older Adult)
- Sex
- 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
Outcomes
Primary Outcomes
Measurement of the diameter of the arterial lumen (µm)
Time Frame: Enrollment
Adaptive optics
Measuring the width of the Kayser-Fleischer ring
Time Frame: Enrollment
OCT-B scan
Corrected visual acuity measurement
Time Frame: Enrollment
ETDRS
Measurement of the central avascular zone (in µm2)
Time Frame: Inclusion
OCT-A
Measurement of the arterial wall section area (µm²)
Time Frame: Enrollment
Adaptive optics
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 capillary density of the deep plexus (in%)
Time Frame: Enrollment
OCT-A
Measurement of the capillary density of the superficial 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
Secondary Outcomes
No secondary outcomes reported