ReviewDocumenta ophthalmologica. Advances in ophthalmology2008
Functional characteristics of patients with retinal dystrophy that manifest abnormal parafoveal annuli of high density fundus autofluorescence; a review and update.
Review in Documenta ophthalmologica. Advances in ophthalmology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 78 papers.
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Who cites it
78 citing papers in PubMed, 143 citations in OpenAlex.
- Effect of Oral Valproic Acid vs Placebo for Vision Loss in Patients With Autosomal Dominant Retinitis Pigmentosa: A Randomized Phase 2 Multicenter Placebo-Controlled Clinical Trial.JAMA ophthalmology · 2018Trial
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- Classification of fundus autofluorescence images based on macular function in retinitis pigmentosa using convolutional neural networks.Japanese journal of ophthalmology · 2025Observational
- Characterization of Macular Fundus Autofluorescence Changes in Patients with Retinitis Pigmentosa.Ophthalmic research · 2025Article
- An unusual pAIR: Anti-PKM2 antibody and occult pancreatic adenocarcinoma.American journal of ophthalmology case reports · 2024Article
- Novel and Previously Known Mutations of theJournal of clinical medicine · 2024Article
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- Detailed Clinical, Ophthalmic, and Genetic Characterization of ADGRV1-Associated Usher Syndrome.American journal of ophthalmology · 2023Article
- Deep phenotypic characterization of the retinal dystrophy in patients with RNU4ATAC-associated Roifman syndrome.Eye (London, England) · 2023Article
- Application of Electrophysiology in Non-Macular Inherited Retinal Dystrophies.Journal of clinical medicine · 2023Review
- Review
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- Primary versus Secondary Elevations in Fundus Autofluorescence.International journal of molecular sciences · 2023Review
- Genetic Characteristics and Long-Term Follow-Up of Slovenian Patients with RPGR Retinal Dystrophy.International journal of molecular sciences · 2023Article
- Morphological and functional parameters in X-linked retinoschisis patients-A multicentre retrospective cohort study.Frontiers in medicine · 2023Article
- Structural evaluation in inherited retinal diseases.The British journal of ophthalmology · 2021Review
- KCNV2-Associated Retinopathy: Detailed Retinal Phenotype and Structural Endpoints-KCNV2 Study Group Report 2.American journal of ophthalmology · 2021Article
- Bisretinoids of the Retina: Photo-Oxidation, Iron-Catalyzed Oxidation, and Disease Consequences.Antioxidants (Basel, Switzerland) · 2021Review
- KCNV2-Associated Retinopathy: Genetics, Electrophysiology, and Clinical Course-KCNV2 Study Group Report 1.American journal of ophthalmology · 2021Article
- Article
18 more citing papers are in PubMed but not listed here.
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeTo examine the presence and functional significance of annular fundus autofluorescence abnormalities in patients with different retinal dystrophies.
methodsEighty one patients were ascertained who had a parafoveal ring of high density on fundus autofluorescence imaging. Sixty two had had a clinical diagnosis of retinitis pigmentosa (RP) or Usher syndrome with normal visual acuity. Others included a case of Leber congenital amaurosis and genetically confirmed cases of cone or cone-rod dystrophy (GUCA1A, RPGR, RIMS1), "cone dystrophy with supernormal rod ERG" (KCNV2) and X-linked retinoschisis (RS1). International-standard full-field and pattern electroretinography (ERG; PERG) were performed. Some patients with rod-cone or cone-rod dystrophy underwent multifocal ERG (mfERG) testing and photopic and scotopic fine matrix mapping (FMM).
resultsIn patients with RP, the radius of the parafoveal ring of high density correlated with PERG P50 (R = 0.83, P < 0.0005, N = 62) and encircled areas of preserved photopic function. In the other patients, AF rings either resembled those seen in RP or encircled an area of central atrophy. Ring radius was inversely related to the PERG P50 component in 4 of 18 cases with a detectable response. FMM showed that arcs of high density were associated with a gradient of sensitivity change.
conclusionsParafoveal rings of high density autofluorescence are a non-specific manifestation of retinal dysfunction that can occur in different retinal dystrophies. Electrophysiology remains essential for accurate diagnosis. The high correlation of autofluorescence with PERG, mfERG and FMM demonstrates that AF abnormalities have functional significance and may help identify suitable patients and retinal areas amenable to future therapeutic intervention.
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