Purpose: Stargardt disease (STGD1) is the most common hereditary macular degeneration and currently no treatments have been approved to interrupt the progression of the disease. The aim of this study was to evaluate the efficacy of photo-biomodulation in patients with Stargardt disease 1 year after treatment, considering as primary outcomes improvements in best corrected visual acuity (BCVA), microperimetry, and pattern electroretinography (PERG) amplitude.
Materials And Methods: Ninety eyes of 45 patients with a diagnosis of Stargardt disease stage 1 were prospectively recruited at the University of Bologna. Selected patients were treated with photo-biomodulation therapy for 1 year for 10 minutes a day, twice a day, and 5 days per week for 12 months. A light emitting diode (LED) of 10 Hz and wavelength 650 nm was used. BCVA and microperimetry were assessed before treatment and 1 year after photo-biomodulation, whereas PERG was assessed at baseline, 1, 3, 6, and 12 months after treatment.
Results: BCVA improved from 0.7 [interquartile range (IQR)=0.6-0.9)] to 0.4 (IQR=0.2-0.5)] after treatment (95% CI=-0.3, -0.35; =<0.001) and PERG negative wave at 35 ms (N35)-positive wave at 50 ms (P50) amplitude improved from 0.9 (IQR=0.74-1.21) to 2.12 (95% CI=1.11, 1.28; <0.001). MP-1 Microperimetry improved from 26.5% (IQR=16.75-40) of median preferred retinal loci (PRL) within an area of 2° of diameter to 38% (IQR=29-50.25; 95% CI=10.50, 14.50; =<0.001); on the other hand, PRL within 4° of diameter raised from 72% (IQR=51.5-80) to 80% (IQR=76-87) after photo-biomodulation (95% CI=4.50, 7.50; <0.001). Also, patients with an improvement of more than 1.205 PERG N35-P50 ratio amplitude at 3 months and more than 1.29 at 6 months tended to present an improvement of more than 0.2 logMar in BCVA at 12 months (=0.28, AUC=0.630; =0.30, AUC=0.636).
Conclusion: BCVA, PERG, and MP-1 significantly improved 1 year after treatment. Also, improvement of PERG N35-P50 amplitude at 3 and 6 months was correlated to BCVA improvement at 1 year, suggesting that PERG could be useful in predicting visual outcome after photo-biomodulation.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8760100 | PMC |
| http://dx.doi.org/10.2147/OPTH.S344378 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Modifiers and their impact on inherited retinal diseases: a review.
Ophthalmic Genet
January 2025
Department of Small Animal Clinical Sciences, Michigan State University, East Lansing, Michigan, USA.
Background: The phenotypic variability of inherited conditions can be due to several factors including environmental, epigenetic, and genetic. One of those genetic factors is the presence of modifying loci which alter the phenotypic expression of a primary disease or phenotype-causing variant. Modifiers are known to affect penetrance, dominance, expressivity, and pleiotropy of disease.
View Article and Find Full Text PDFStargardt disease is a currently untreatable, inherited neurodegenerative disease that leads to macular degeneration and blindness due to loss-of-function mutations in the ABCA4 gene. We have designed a dual adeno-associated viral vector encoding a split-intein adenine base editor to correct the most common mutation in ABCA4 (c.5882G>A, p.
View Article and Find Full Text PDFQuercetin Alleviates All--Retinal-Induced Photoreceptor Apoptosis and Retinal Degeneration by Inhibiting the ER Stress-Related PERK Signaling.
Int J Mol Sci
December 2024
Fujian Provincial Key Laboratory of Ophthalmology and Visual Science, Fujian Engineering and Research Center of Eye Regenerative Medicine, Eye Institute of Xiamen University, School of Medicine, Xiamen University, Xiamen 361102, China.
All--retinal (atRAL)-induced photoreceptor atrophy and retinal degeneration are hallmark features of dry age-related macular degeneration (AMD) and Stargardt disease type 1 (STGD1). The toxicity of atRAL is closely related to the generation of reactive oxygen species (ROS). Quercetin, a natural product, is known for its potent antioxidant properties; however, its effects in mitigating atRAL-mediated retinal damage remains unclear.
View Article and Find Full Text PDFRescue of Aberrant Splicing Caused by a Novel Complex Deep-intronic Allele.
Genes (Basel)
November 2024
Institute of Medical Molecular Genetics, University of Zurich, 8952 Schlieren, Switzerland.
Stargardt disease (STGD1) is an autosomal recessive disorder caused by pathogenic variants in that affects the retina and is characterised by progressive central vision loss. The onset of disease manifestations varies from childhood to early adulthood. Whole exome (WES), whole gene, and whole genome sequencing (WGS) were performed for a patient with STGD1.
View Article and Find Full Text PDF12-year cumulative incidence rate of rare retinal diseases: a nationwide study in Korea.
Eye (Lond)
January 2025
Department of Ophthalmology, Chung-Ang University, College of Medicine, Seoul, South Korea.
Purpose: Understanding the incidence of rare diseases is important in establishing a proper public health care system and setting target diseases in medical research. Herein, we report the 12-year cumulative incidence of seven rare ocular diseases of the retina in South Korea.
Methods: We analysed clinical records of 1,126,250 South Korean population during 2006~2019.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!