Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy.
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AuthorJenny, Laura A
Kim, Angela H
Levi, Sarah R
Greenstein, Vivienne C
Tsang, Stephen H
Journal titleScientific reports
Publication Begin page14706
MetadataShow full item record
AbstractBietti crystalline dystrophy (BCD) is an ultra-rare orphan disorder that can lead to blindness. Because of the variable rates of progression of the disease, it is necessary to identify suitable outcome measurements for tracking progression in BCD. A retrospective analysis of patients with a clinical and genetic diagnosis of BCD was conducted. Four measurements of spectral domain-optical coherence tomography were compared to patients' best corrected visual acuity. We observed that patients with higher measurements of foveolar thickness, choroidal thickness in the foveolar region, ellipsoid zone band length and the outer nuclear layer + area, had on average better visual acuity. Future studies are needed to validate the structural-functional correlations we observed in BCD and to propose a sensitive and clinically meaningful outcome measurement for tracking this rare, variable disease.
CitationJenny LA, Liu PK, Kolesnikova M, Duong J, Kim AH, Levi SR, Greenstein VC, Tsang SH. Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy. Sci Rep. 2022 Aug 29;12(1):14706. doi: 10.1038/s41598-022-16563-y. PMID: 36038562; PMCID: PMC9424222.
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- Creative Commons
Except where otherwise noted, this item's license is described as © 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.
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