title: The effect of light deprivation in patients with Stargardt disease.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
authors: Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
authors: Michel M
authors: MM
authors: Teussink
authors: Department of Ophthalmology, Columbia University, New York, New York.
authors: Michele D
authors: MD
authors: Lee
authors: Department of Ophthalmology, New York University School of Medicine, New York, New York.
authors: R Theodore
authors: RT
authors: Smith
authors: Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
authors: Ramon A C
authors: RA
authors: van Huet
authors: Erasmus University Medical Center, Rotterdam, Netherlands.
authors: Caroline C
authors: CC
authors: Klaver
authors: Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
authors: B Jeroen
authors: BJ
authors: Klevering
authors: Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands. Electronic address: Thomas.Theelen@radboudumc.nl.
authors: Thomas
authors: T
authors: Theelen
authors: Department of Ophthalmology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands.
authors: Carel B
authors: CB
authors: Hoyng
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000293:Adolescent; D000328:Adult; D002648:Child; D003261:Contact Lenses; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008027:Light; D008062:Lipofuscin / Q000378:metabolism; D008268:Macular Degeneration / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D011835:Radiation Protection / Q000295:instrumentation / Q000379:methods*; D055213:Retinal Pigment Epithelium / Q000473:pathology; D012189:Retrospective Studies; D012683:Sensory Deprivation; D000080362:Stargardt Disease; D055815:Young Adult
chemical_list: C056475:ABCA4 protein, human; D018528:ATP-Binding Cassette Transporters; D008062:Lipofuscin