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Am J Ophthalmol. 2015 May;159(5):964-72.e2. doi: 10.1016/j.ajo.2015.02.004. Epub 2015 Feb 11.

The effect of light deprivation in patients with Stargardt disease.

Author information

1
Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
2
Department of Ophthalmology, Columbia University, New York, New York.
3
Department of Ophthalmology, New York University School of Medicine, New York, New York.
4
Erasmus University Medical Center, Rotterdam, Netherlands.
5
Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands. Electronic address: Thomas.Theelen@radboudumc.nl.

Abstract

PURPOSE:

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.

DESIGN:

Longitudinal, retrospective, interventional case series.

METHODS:

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.

RESULTS:

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.

CONCLUSIONS:

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.

PMID:
25681002
DOI:
10.1016/j.ajo.2015.02.004
[Indexed for MEDLINE]
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