Diagnostic impact of anterior segment angiography of limbal stem cell insufficiency in PAX6 ‐related aniridia

PAX6 is a master gene of ocular development and postnatal ocular equilibrium. Congenital aniridia is the hallmark of PAX6 gene haploinsufficiency (Chr. 11 p. 13), but PAX6‐associated aniridia is a profound, progressive pan‐ocular developmental disorder often leading to blindness. There is congenital visual impairment with advancing loss of vision mainly due to secondary glaucoma and to corneal blindness caused by limbal stem cell insufficiency (LSCI). LSCI leads to ARK (aniridia‐related keratopathy), which typically develops in four stages. Incipient LSCI with vessels starting to grow into the cornea can be imaged by fluorescein anterior segment angiography, which enables fine vessels to be more easily detected than by routine slit lamp examination, especially in patients with nystagmus. Thus, clinical stage 1 ARK is often diagnosed at stage 2 by angiography. Corneal neovascularizations often start at the 12 and 6 positions and subsequently progress circumferentially, not at the 3 and 9 positions as previously believed. Anterior segment angiography can provide an easily standardizable tool for monitoring progress, treatment‐induced regress or stabilization of ARK. Especially in children, angiography could be used to monitor new treatment regimens for reducing LSCI. Angiography could enable treatment to begin earlier to preserve corneal hemostasis. In addition, the fact that vascularization often starts at the subpalpebral 6 and 12 positions as opposed to the 3 and 9 p...
Source: Clinical Anatomy - Category: Anatomy Authors: Tags: Original Communication Source Type: research

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Authors: Zhang X, Tenerelli K, Wu S, Xia X, Yokota S, Sun C, Galvao J, Venugopalan P, Li C, Madaan A, Goldberg JL, Chang KC Abstract BACKGROUND: Glaucoma, the number one cause of irreversible blindness, is characterized by the loss of retinal ganglion cells (RGCs), which do not regenerate in humans or mammals after cell death. Cell transplantation provides an opportunity to restore vision in glaucoma, or other optic neuropathies. Since transplanting primary RGCs from deceased donor tissues may not be feasible, stem cell-derived RGCs could provide a plausible alternative source of donor cells for transplant. OBJ...
Source: Restorative Neurology and Neuroscience - Category: Neurology Tags: Restor Neurol Neurosci Source Type: research
AbstractGlaucoma is the second leading cause of blindness worldwide. Even though significant advances have been made in its management, currently available antiglaucoma therapies suffer from considerable drawbacks. Typically, the success and efficacy of glaucoma medications are undermined by their limited bioavailability to target tissues and the inadequate adherence demonstrated by patients with glaucoma. The latter is due to a gradual decrease in tolerability of lifelong topical therapies and the significant burden to patients of prescribed stepwise antiglaucoma regimens with frequent dosing which impact quality of life....
Source: Advances in Therapy - Category: Drugs & Pharmacology Source Type: research
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Source: International Ophthalmology - Category: Opthalmology Source Type: research
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Source: Acta Microbiologica et Immunologica Hungarica - Category: Microbiology Authors: Tags: Acta Microbiol Immunol Hung Source Type: research
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(University College London) Living in a more polluted area is associated with a greater likelihood of having glaucoma, a debilitating eye condition that can cause blindness, finds a new UCL-led study in the UK. People in neighbourhoods with higher amounts of fine particulate matter pollution were at least 6% more likely to report having glaucoma than those in the least-polluted areas, according to the findings published in Investigative Ophthalmology&Visual Science.
Source: EurekAlert! - Biology - Category: Biology Source Type: news
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Source: SUNY Upstate Medical - Category: Universities & Medical Training Tags: News Source Type: news
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Source: The British Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Br J Ophthalmol Source Type: research
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Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Article Source Type: research
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