Study of Retinal Vascular Parameters in Patients With Wilson's Disease
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 124
- 试验地点
- 2
- 主要终点
- Measurement of the diameter of the arterial lumen (µm)
研究概览
简要总结
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.).
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Other
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —(Adult, Older Adult)
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Wilson's disease with Ferenci score ≥4
排除标准
- •Neurological or behavioral handicap that does not allow for ophthalmological examinations
- •Hepatic decompensation that does not allow for ophthalmological examinations
- •Pregnant or breastfeeding woman
结局指标
主要结局
Measurement of the diameter of the arterial lumen (µm)
时间窗: Enrollment
Adaptive optics
Measuring the width of the Kayser-Fleischer ring
时间窗: Enrollment
OCT-B scan
Corrected visual acuity measurement
时间窗: Enrollment
ETDRS
Measurement of the central avascular zone (in µm2)
时间窗: Inclusion
OCT-A
Measurement of the arterial wall section area (µm²)
时间窗: Enrollment
Adaptive optics
Intraocular pressure measurement (mmHg)
时间窗: Enrollment
Measurement of the thickness of the retinal nerve fibers layer (RNFL)
时间窗: Enrollment
OCT-B
Measurement of the capillary density of the deep plexus (in%)
时间窗: Enrollment
OCT-A
Measurement of the capillary density of the superficial plexus (in%)
时间窗: Enrollment
OCT-A
Measurement of photoreceptor density (per mm2)
时间窗: Enrollment
Adaptive optics
Measurement of wall to lumen ratio (in %)
时间窗: Enrollment
Adaptive optics
次要结局
未报告次要终点
