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Coats-Like reaction post-vitreoretinal surgery for PDR managed with laser photocoagulation and adjunctive intravitreal steroids- a case report.
BMC Ophthalmol
January 2025
Dept. of Retina and Vitreous, Narayana Nethralaya, #121/C, 1st R Block, Chord Road, Rajaji Nagar, Bengaluru, 560010, India.
Purpose: To report a rare case of a Coats-like response developing after vitreoretinal surgery for proliferative diabetic retinopathy (PDR) and its successful management with retinal laser photocoagulation and adjunctive intravitreal steroids.
Case Description: A 52-year-old woman with a five-year history of type 2 diabetes mellitus and hypertension presented with decreased vision in the left eye (counting fingers at 1 m). Examination revealed high-risk PDR in both eyes, with a subtotal macula-off combined retinal detachment in the left eye.
Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder caused by mutations in the bone morphogenetic protein signaling pathway, leading to arteriovenous malformations. While previously thought to share molecular and cellular dysregulation, this study reveals highly distinct mechanisms depending on whether mutations occur in Alk1 or SMAD4. Loss of SMAD4 enhances endothelial cell responses to flow, including flow-regulated transcription and cell migration against blood flow, causing excessive pruning of capillaries and the formation of single large shunts.
View Article and Find Full Text PDFPerifoveal vascular anomalous complex and telangiectatic capillaries: An overview of two entities potentially sharing a common pathophysiology.
Surv Ophthalmol
January 2025
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Division of head and neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address:
Focal capillary ectasia in the macular region can manifest in distinct clinical scenarios, which can be categorized into 2 main entities: perifoveal vascular anomalous complex (PVAC) and telangiectatic capillaries (TelCaps). PVAC represents a primary, idiopathic condition, whereas TelCaps occur secondary to underlying vascular disorders, including diabetic macular edema and retinal vein occlusion. We provide a comprehensive analysis of these 2 entities, encompassing their clinical presentations, multimodal imaging findings, histological evidence, and differential diagnosis from other retinal microvascular abnormalities, such as Type 1 macular telangiectasia, adult-onset Coats disease, Type 3 macular neovascularization in age-related macular degeneration, and retinal arterial macroaneurysms.
View Article and Find Full Text PDFA phase II double-blind multicentre, placebo-controlled trial to assess the efficacy and safety of alpelisib (BYL719) in paediatric and adult patients with Megalencephaly-CApillary malformation Polymicrogyria syndrome (MCAP): the SESAM study protocol.
BMJ Open
December 2024
INSERM UMR1231 Génétique des Anomalies du Développement (GAD), Université de Bourgogne, Dijon, France.
Introduction: The megalencephaly capillary malformation polymicrogyria (MCAP syndrome) results from mosaic gain-of-function variants. The main clinical features are macrocephaly, somatic overgrowth, neurodevelopmental delay and brain anomalies. Alpelisib (Vijoice) is a recently FDA-approved PI3Kα-specific inhibitor for patients with PIK3CA-related overgrowth spectrum (PROS).
View Article and Find Full Text PDFOCT Angiography characteristics of Bilateral Mactel type 1: revealing the Aneurysmo occlusive variety.
Retin Cases Brief Rep
December 2024
Department of VitreoRetina, Aditya Birla SankaraNethralaya,147, Mukundapur, E.M. Bypass, Kolkata-700 099, West Bengal, India.
Purpose: To report OCTA (Optical coherence tomography angiography) characteristics of bilateral MacTel type 1 with capillary obliteration.
Method: Colour fundus photo (CFP), OCT angiography (OCTA), Fundus fluorescein angiography (FFA), Fundus autofluorescence (FAF).
Result: We describe three cases of bilateral MacTel Type 1 with a special emphasis on OCTA characteristics.
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