Teussink Michel M, Lee Michele D, Smith R Theodore, van Huet Ramon A C, Klaver Caroline C, Klevering B Jeroen, Theelen Thomas, Hoyng Carel B
Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
Department of Ophthalmology, Columbia University, New York, New York.
Am J Ophthalmol. 2015 May;159(5):964-72.e2. doi: 10.1016/j.ajo.2015.02.004. Epub 2015 Feb 11.
To investigate whether long-term protection from light exposure affects the rate of disease progression in patients with autosomal recessive Stargardt disease (STGD1), measured using fundus autofluorescence imaging.
Longitudinal, retrospective, interventional case series.
Five patients with Stargardt disease protected 1 eye from light exposure by applying a black contact lens during waking hours for ≥12 months. Disease progression was followed by performing autofluorescence imaging at semi-regular intervals. Longitudinal changes in autofluorescence were studied by evaluating areas of decreased autofluorescence and areas of increased autofluorescence as a measure of retinal pigment epithelium damage and lipofuscin accumulation, respectively.
We observed less progression of decreased autofluorescence in 4 out of 5 light-protected eyes relative to their respective nonprotected eyes. The progression of increased autofluorescence, on the other hand, was highly variable and did not respond consistently to treatment.
Areas of decreased autofluorescence may serve as a useful biomarker for measuring the progression of Stargardt disease. The reduced progression of decreased autofluorescence in the light-protected eyes suggests that light deprivation might be beneficial in patients with Stargardt disease.
使用眼底自发荧光成像技术，研究长期避光是否会影响常染色体隐性遗传性Stargardt病（STGD1）患者的疾病进展速度。
纵向、回顾性、干预性病例系列研究。
5例Stargardt病患者在清醒时间佩戴黑色隐形眼镜≥12个月，对1只眼睛进行避光保护。通过定期进行自发荧光成像来跟踪疾病进展。分别通过评估自发荧光降低区域和自发荧光增加区域，研究自发荧光的纵向变化，以此作为视网膜色素上皮损伤和脂褐素积累的指标。
我们观察到，5只受光保护眼中的4只相对于各自未受保护的眼睛，自发荧光降低区域的进展较慢。另一方面，自发荧光增加区域的进展变化很大，对治疗没有一致的反应。
自发荧光降低区域可能是衡量Stargardt病进展的有用生物标志物。受光保护眼的自发荧光降低区域进展减缓，表明避光可能对Stargardt病患者有益。
