Multimodal imaging in best vitelliform macular dystrophy

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Multimodal analysis of the progression of Best vitelliform macular dystrophy

PURPOSE To investigate the multimodal morphological features in the different stages of Best vitelliform macular dystrophy (VMD) in subjects harboring mutations in the BEST1 gene, and their changes during the progression of the disease. METHODS In this retrospective observational study performed between January 2007 and December 2012, 21 patients (42 eyes) with Best VMD from eight families wi...

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Best Vitelliform Macular Dystrophy In Afghan Twins.

Best vitelliform macular dystrophy is the second most common type of hereditary fundus dystrophies. Our case report describes twin brothers of Afghan family who developed Best vitelliform macular dystrophy. One of them developed choroidal neovascularization in one eye which was treated with single intravitreal injection of bevacizumab. The patient showed stable vision and did not have any recur...

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Photoreceptor Inner Segment Morphology in Best Vitelliform Macular Dystrophy.

PURPOSE To characterize outer retina structure in best vitelliform macular dystrophy (BVMD) and to determine the effect of macular lesions on overlying and adjacent photoreceptors. METHODS Five individuals with BVMD were followed prospectively with spectral domain optical coherence tomography and confocal and nonconfocal split-detector adaptive optics scanning light ophthalmoscopy (AOSLO). Th...

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Dark adaptation in patients with Best vitelliform macular dystrophy.

Psychophysical dark adaptation studies were performed in six patients with Best vitelliform macular dystrophy (BVMD) using a Goldmann-Weekers dark adaptometer. Prebleach thresholds were determined before obtaining a postbleach full recovery curve. Unlike patients with Stargardt macular dystrophy, all patients with BVMD showed a normal time to reach their baseline dark adapted thresholds after b...

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A model of best vitelliform macular dystrophy in rats.

PURPOSE The VMD2 gene, mutated in Best macular dystrophy (BMD) encodes bestrophin, a 68-kDa basolateral plasma membrane protein expressed in retinal pigment epithelial (RPE) cells. BMD is characterized by a depressed light peak (LP) in the electro-oculogram. Bestrophin is thought to be the Cl channel that generates the LP. The goal was to generate an animal model of BMD and to determine the eff...

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ژورنال

عنوان ژورنال: Annals of Eye Science

سال: 2019

ISSN: 2520-4122

DOI: 10.21037/aes.2019.08.04