ArticleAmerican journal of ophthalmology2019
Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography.
Article in American journal of ophthalmology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 37 citations in OpenAlex.
- Three-Year Safety Results of SAR422459 (EIAV-ABCA4) Gene Therapy in Patients With ABCA4-Associated Stargardt Disease: An Open-Label Dose-Escalation Phase I/IIa Clinical Trial, Cohorts 1-5.American journal of ophthalmology · 2022Trial
- Dysregulated DNA Methylation inInternational journal of molecular sciences · 2025Article
- Bisretinoids as a Source of Early Photoreceptor Pathology in Stargardt Disease.Ophthalmic research · 2025Review
- Stargardt macular dystrophy and therapeutic approaches.The British journal of ophthalmology · 2024Review
- Multimodal in-vivo maps as a tool to characterize retinal structural biomarkers for progression in adult-onset Stargardt disease.Frontiers in ophthalmology · 2024Article
- Optical Coherence Tomography and Optical Coherence Tomography Angiography in Pediatric Retinal Diseases.Diagnostics (Basel, Switzerland) · 2023Review
- The Role of the Choroid in Stargardt Disease.International journal of molecular sciences · 2022Review
- Structural evaluation in inherited retinal diseases.The British journal of ophthalmology · 2021Review
- Localized Structural and Functional Deficits in a Nonhuman Primate Model of Outer Retinal Atrophy.Investigative ophthalmology & visual science · 2021Article
- Assessment of Detailed Photoreceptor Structure and Retinal Sensitivity in Diabetic Macular Ischemia Using Adaptive Optics-OCT and Microperimetry.Investigative ophthalmology & visual science · 2021Article
- Stargardt disease: Multimodal imaging: A review.Clinical & experimental ophthalmology · 2021Review
- Artifacts and artifact removal in optical coherence tomographic angiography.Quantitative imaging in medicine and surgery · 2021Review
- Clinical spectrum, genetic complexity and therapeutic approaches for retinal disease caused by ABCA4 mutations.Progress in retinal and eye research · 2020Review
- Analysis of retinal sublayer thicknesses and rates of change in ABCA4-associated Stargardt disease.Scientific reports · 2020Observational
- Evaluation of the different thresholding strategies for quantifying choriocapillaris using optical coherence tomography angiography.Quantitative imaging in medicine and surgery · 2020Article
- Repeatability of binarization thresholding methods for optical coherence tomography angiography image quantification.Scientific reports · 2020Article
- Retinal Glial and Choroidal Vascular Pathology in Donors Clinically Diagnosed With Stargardt Disease.Investigative ophthalmology & visual science · 2020Article
- Advancing Clinical Trials for Inherited Retinal Diseases: Recommendations from the Second Monaciano Symposium.Translational vision science & technology · 2020Review
- Progressive Choriocapillaris Impairment in ABCA4 Maculopathy Is Secondary to Retinal Pigment Epithelium Atrophy.Investigative ophthalmology & visual science · 2020Article
- Macular dystrophies: clinical and imaging features, molecular genetics and therapeutic options.The British journal of ophthalmology · 2020Review
Corrections and comments
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Authors and funding
14 authors at 2 institutions in 3 countries.
Funding
Abstract
purposeThis study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD).
designProspective cross-sectional study.
methodsThis study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated.
resultsThe total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P < 0.0001) by a 1.6:1 ratio (r
conclusionsPhotoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.
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