Clinical trial NCT04408300: Study of Retinal Vascular Parameters in Patients With Wilson's Disease
Official title: Study of Retinal Vascular Parameters in Patients With Wilson's Disease
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Wilson Disease
Keywords: Optical Coherence Tomography
Adaptive optics
Lead sponsor: Fondation Ophtalmologique Adolphe de Rothschild
Sponsor class: NETWORK
First posted: 2020-05-29
Enrollment: 124
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: OTHER

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.).


Eligibility criteria: 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

Primary outcomes:
measure: Measuring the width of the Kayser-Fleischer ring
timeFrame: Enrollment
measure: Corrected visual acuity measurement
timeFrame: Enrollment
measure: Intraocular pressure measurement (mmHg)
timeFrame: Enrollment
measure: Measurement of the thickness of the retinal nerve fibers layer (RNFL)
timeFrame: Enrollment
measure: Measurement of the central avascular zone (in µm2)
timeFrame: Inclusion
measure: Measurement of the capillary density of the superficial plexus (in%)
timeFrame: Enrollment
measure: Measurement of the capillary density of the deep plexus (in%)
timeFrame: Enrollment
measure: Measurement of photoreceptor density (per mm2)
timeFrame: Enrollment
measure: Measurement of wall to lumen ratio (in %)
timeFrame: Enrollment
measure: Measurement of the arterial wall section area (µm²)
timeFrame: Enrollment
measure: Measurement of the diameter of the arterial lumen (µm)
timeFrame: Enrollment
Secondary outcomes:
Other outcomes:
Locations:
city: Paris
country: France
facility: Hôpital Fondation A. de Rothschild
Study officials:
affiliation: Fondation A. de Rothschild
name: Aurélia POUJOIS, MD
role: PRINCIPAL_INVESTIGATOR
References: