Publications by authors named "C B Hoyng"
Syndromic Retinitis Pigmentosa.
Prog Retin Eye Res
December 2024
Retinitis pigmentosa (RP) is a progressive inherited retinal dystrophy, characterized by the degeneration of photoreceptors, presenting as a rod-cone dystrophy. Approximately 20-30% of patients with RP also exhibit extra-ocular manifestations in the context of a syndrome. This manuscript discusses the broad spectrum of syndromes associated with RP, pathogenic mechanisms, clinical manifestations, differential diagnoses, clinical management approaches, and future perspectives.
View Article and Find Full Text PDFIntroduction: Half-dose photodynamic therapy (HD-PDT) with verteporfin is the mainstay treatment in central serous chorioretinopathy (CSC). Since 2021, there is a worldwide shortage of verteporfin. This called for adjustments of daily practice.
View Article and Find Full Text PDFArticle Synopsis
- Age-related macular degeneration (AMD) is a complex eye disease influenced by both genetic factors and involves a subtype called reticular pseudodrusen (RPD), which increases the risk of severe vision loss.* -
- A genome-wide association study compared genetic data from individuals with AMD and/or RPD to controls, finding significant associations specifically on chromosome 10, while chromosome 1 did not show this correlation in RPD cases.* -
- The chromosome 10 region includes a long non-coding RNA related to retinal health, highlighting its potential role in retinal thickness and influencing the outer segment of photoreceptors.*
Article Synopsis
- The study aims to evaluate how functional and structural assessments can serve as endpoints in clinical trials for retinal degeneration linked to USH2A mutations.
- Participants with specific visual capabilities underwent various eye tests over four years, focusing on understanding changes in their vision.
- Findings indicated that certain tests were more sensitive to detecting changes, influencing the design of future clinical trials related to this condition.
Purpose: The purpose of this study was to develop a deep learning algorithm for detecting and quantifying incomplete retinal pigment epithelium and outer retinal atrophy (iRORA) and complete retinal pigment epithelium and outer retinal atrophy (cRORA) in optical coherence tomography (OCT) that generalizes well to data from different devices and to validate in an intermediate age-related macular degeneration (iAMD) cohort.
Methods: The algorithm comprised a domain adaptation (DA) model, promoting generalization across devices, and a segmentation model for detecting granular biomarkers defining iRORA/cRORA, which are combined into iRORA/cRORA segmentations. Manual annotations of iRORA/cRORA in OCTs from different devices in the MACUSTAR study (168 patients with iAMD) were compared to the algorithm's output.