Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy
Abstract Bietti 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...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Nature Portfolio
2022-08-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-022-16563-y |
| _version_ | 1811297360250142720 |
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| author | Laura A. Jenny Pei-Kang Liu Masha Kolesnikova Jimmy Duong Angela H. Kim Sarah R. Levi Vivienne C. Greenstein Stephen H. Tsang |
| author_facet | Laura A. Jenny Pei-Kang Liu Masha Kolesnikova Jimmy Duong Angela H. Kim Sarah R. Levi Vivienne C. Greenstein Stephen H. Tsang |
| author_sort | Laura A. Jenny |
| collection | DOAJ |
| description | Abstract Bietti 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. |
| first_indexed | 2024-04-13T06:03:06Z |
| format | Article |
| id | doaj.art-54c7dd531cb745aa9472ea5dc3bc16ce |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-04-13T06:03:06Z |
| publishDate | 2022-08-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-54c7dd531cb745aa9472ea5dc3bc16ce2022-12-22T02:59:21ZengNature PortfolioScientific Reports2045-23222022-08-0112111010.1038/s41598-022-16563-yFoveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophyLaura A. Jenny0Pei-Kang Liu1Masha Kolesnikova2Jimmy Duong3Angela H. Kim4Sarah R. Levi5Vivienne C. Greenstein6Stephen H. Tsang7Edward S. Harkness Eye Institute, New York-Presbyterian HospitalJonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia UniversityEdward S. Harkness Eye Institute, New York-Presbyterian HospitalEdward S. Harkness Eye Institute, New York-Presbyterian HospitalEdward S. Harkness Eye Institute, New York-Presbyterian HospitalEdward S. Harkness Eye Institute, New York-Presbyterian HospitalEdward S. Harkness Eye Institute, New York-Presbyterian HospitalEdward S. Harkness Eye Institute, New York-Presbyterian HospitalAbstract Bietti 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.https://doi.org/10.1038/s41598-022-16563-y |
| spellingShingle | Laura A. Jenny Pei-Kang Liu Masha Kolesnikova Jimmy Duong Angela H. Kim Sarah R. Levi Vivienne C. Greenstein Stephen H. Tsang Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy Scientific Reports |
| title | Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy |
| title_full | Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy |
| title_fullStr | Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy |
| title_full_unstemmed | Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy |
| title_short | Foveolar thickness as potential standardized structural outcome measurement in studies of Bietti crystalline dystrophy |
| title_sort | foveolar thickness as potential standardized structural outcome measurement in studies of bietti crystalline dystrophy |
| url | https://doi.org/10.1038/s41598-022-16563-y |
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