Kellner, Simone and Kellner, Ulrich and Weber, Bernhard H. F. and Fiebig, Britta and Weinitz, Silke and Ruether, Klaus (2009) Lipofuscin- and Melanin-related Fundus Autofluorescence in Patients with ABCA4-associated Retinal Dystrophies. AMERICAN JOURNAL OF OPHTHALMOLOGY, 147 (5). pp. 895-902. ISSN 0002-9394, 1879-1891
Full text not available from this repository. (Request a copy)Abstract
PURPOSE: To compare melanin-related near-infrared fundus autofluorescence (NIA; excitation 787 nm, emission > 800 nm) to lipofuscin-related fundus autofluorescence (FAF; excitation 488 nm, emission > 500 nm) in patients with retinal dystrophies associated with ABCA4 gene mutations (ABCA4-RD). DESIGN: Observational case series. METHODS: Sixteen consecutive patients with ABCA4-RD diagnosed in one institution were included. FAF and NIA imaging were performed with a confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph 2; Heidelberg Engineering, Heidelberg, Germany). The pattern and size of retinal pigment epithelial (RPE) alterations detected with FAF and NIA were evaluated. 0 RESULTS: FAF and NIA alterations were detected in all patients. In 7 of 16 patients, the alterations progressed beyond the vascular arcades, and in 9 of 16, they were confined to the macula. Spots of increased NIA (4/16) were less frequent compared with spots of increased FAF (15/16). Confluent patches of reduced NIA were frequent (12/16), and severely reduced NIA was observed in 3 cases. Areas with reduced NIA corresponded to either increased or reduced FAF. Preservation of subfoveal FAF or NIA corresponded to visual acuity >= 0.4. Abnormalities detected with NIA were more extensive or more severe compared to FAF in 15 of 16 patients. CONCLUSION: Patterns of FAF and NIA indicate different involvement of lipofuscin and melanin and their derivates in the pathophysiologic process of ABCA4-RD. NIA imaging provides a noninvasive in vivo visualization of RPE abnormalities that may precede FAF alterations during the degenerative process. Combined FAF and NIA imaging will provide further insight in the development of ABCA4-RD and could help to monitor future therapeutic interventions. (Am J Ophthalmol 2009; 147:895-902. (c) 2009 by Elsevier Inc. All rights reserved.)
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | PIGMENT EPITHELIAL-CELLS; ABCA4 GENE; IN-VIVO; MACULAR DEGENERATION; STARGARDT-DISEASE; FLAVIMACULATUS; A2E; FLUORESCENCE; MUTATIONS; LIGHT; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Humangenetik |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 16 Sep 2020 04:25 |
| Last Modified: | 16 Sep 2020 04:25 |
| URI: | https://pred.uni-regensburg.de/id/eprint/28990 |
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