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Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Grosspoetzl, M; Riedl, R; Schliessleder, G; Hu, ZJ; Michaelides, M; Sadda, S; Birch, D; Issa, PC; Wedrich, A; Seidel, G; Scholl, HPN; Strauss, RW.
The Progression of PROM1-associated retinal degeneration as determined by spectral-domain optical coherence tomography over a 24-months period.
Am J Ophthalmol. 2023;
Doi: 10.1016/j.ajo.2023.11.010
Web of Science
PubMed
FullText
FullText_MUG
- Leading authors Med Uni Graz
- Großpötzl Manuel
- Strauß Rupert
- Co-authors Med Uni Graz
- Riedl Regina
- Schließleder Gernot
- Seidel Gerald
- Wedrich Andreas
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- Abstract:
- PURPOSE: To evaluate the progression of atrophy as determined by spectral-domain optical coherence tomography (SD-OCT) in patients with molecularly confirmed PROM1-associated retinal degeneration (RD) over a 24 months period. DESIGN: International, multicenter, prospective case series. METHODS: A total of 13 eyes (13 patients) affected with PROM1-associated RD were enrolled at five sites and SD-OCT images were obtained at baseline and after 24 months. Loss of mean thickness (MT) and intact area were estimated after semi-automated segmentation for the following individual retinal layers in the central subfield (CS), inner ring and outer ring of the ETDRS grid: retinal pigment epithelium (RPE), outer segments (OS), inner segments (IS), outer nuclear layer (ONL), inner retina (IR), and total retina (TR). RESULTS: Statistically significant losses of thickness of RPE and TR were detected in the CS and inner ring and of ONL and IS in the outer ring (all p<0,05); a statistically significant decrease of intact area of RPE and IS was observed in the inner ring and of ONL in the outer ring (all p<0,05); the change in MT and the intact area of the other layers showed a trend of decline over an observational period of 24 months. CONCLUSIONS: Significant thickness losses could be detected in outer retinal layers by SD-OCT over a 24 months period in patients with PROM1-associated retinal degeneration. Loss of thickness and/or intact area of such layers may serve as potential endpoints for clinical trials that aim to slow down the disease progression of PROM1-associated retinal degeneration.