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临床试验/NCT04408300
NCT04408300已完成不适用

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

Fondation Ophtalmologique Adolphe de Rothschild2 个研究点 分布在 1 个国家目标入组 124 人开始时间: 2020年6月4日最近更新:
适应症

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

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

研究者

申办方类型
Network
责任方
Sponsor

研究点 (2)

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