Leber’s hereditary optic neuropathy (LHON) is a relatively common, rapidly progressing inherited optic neuropathy wherein LHON-affected eyes undergo optic nerve atrophy due to retinal ganglion cell (RGC) loss. It is a maternally inherited (or sporadic) mitochondrial disorder caused primarily by mutations in genes that encode components of respiratory complex (RC)1 in mitochondria. Mitochondrial deficiency of RC1 compromises ATP production and oxidative stress management in RGCs. The most common LHON-causing mutations are 11778G>A, 3460G>A, and 14484T>C point mutations in MT-ND4, MT-ND1, and MT-ND6. The unusually high mitochondrial load of RGCs makes them particularly sensitive to these mutations. Patients with LHON may be prescribed ubiquinone (a component of RC3) or idebenone, a ubiquinone analogue with enhanced bioavailability to act downstream of RC1. The challenge of accessing the inner mitochondrial membrane with gene therapy for LHON, and other mitochondrial diseases, may be overcome by incorporation of a specific mitochondrion-targeting sequence (MTS) that enables allotropic expression of a nucleus-transcribed ND4 transgene. Because LHON penetrance is incomplete among carriers of the aforementioned mutations, identification of environmental factors, such as heavy smoking, that interact with genetics in the phenotypic expression of LHON may be helpful toward preventing or delaying disease development. LHON has become a model for mitochondrial and neurogenerative diseases owing to it having a clearly identified genetic cause and its early onset and rapid progression characteristics. Hence, LHON studies and genetic treatment advances may inform research of other diseases.
Present investigation deals with a pilot study in which a new technique for applying mitomycin C directly onto the ablated corneal surface in the form of an injector-administered bubble. Here, a technique was introduced and the observations made with this technique in this pilot study involving 125 patients who received wave front bilateral photorefractive keratectomy. Mitomycin C was administered using the common minisponge application technique in one eye and mitomycin C was administered using the new bubble technique in the other eye. Photorefractive keratectomy presentation was compared between the minisponge-treated and bubble-treated eyes. Visual parameters such as visual acuity, spherical refractive error and cylindrical refractive error were similar in eyes across the two treatments before and after wave front photorefractive keratectomy. None of the minisponge-treated or bubble-treated eyes developed clinically evident photorefractive keratectomy haze. Compared to the minisponge-treated eyes, bubbletreated eyes showed faster healing (3 d vs. 5 d for contact lens bandage removal) and faster visual recovery (≤5 d vs. 7-10 d). In conclusion, the new bubble technique for applying mitomycin C after wave front photorefractive keratectomy provided a safe, effective and time-saving alternative to the standard mini sponge mitomycin C application technique.
ÖzRefraktif cerrahi sonrası gelişen diffüz lameller keratit, çoğunlukla unilateral (tek gözde), görme ekseninden uzak, periferal yerleşimli küçük gri-beyaz korneal noktacıklar şeklinde ortaya çıkan bir komplikasyon tablosudur. Başlangıç evrelerde farkına varılmaz ve uygun tedavi ile önlem alınmazsa, ileride ciddi görme kaybına neden olabilmektedir. Özellikle aktif ve genç nüfusun, gözlük ve lens bağımlılığından kurtulmak için tercih ettiği refraktif cerrahi gün geçtikçe daha yoğun bir şekilde uygulanmaktadır. Bu yazıda; ilerleyici ve geri dönüşümsüz kornea ve görme hasarına neden olabilecek, diffüz lameller keratitin, erken tanısı ve tedavisi ile ileride gelişebilecek kalıcı görme kaybının önlenmesi için güncel yaklaşımların ortaya konulması amaçlanmıştır.Anahtar sözcükler : Refraktif cerrahi, diffüz lameller kerattit, korneanın kalıcı nekrozu Diffuse lamella keratitis: diagnosis and treatment methods AbstractDeveloping after refractive surgery, Diffuse Lamellar Keratitis is a complication that appears as small gray-white corneal spots in the periphery. It is mostly unilateral (one-eye) and distant to the visual axis. If it goes unnoticed at initial stages, and if appropriate treatment is not applied, it may cause severe visual impairment. Refractive surgery is especially preferred by active and young population to be released of glasses and lens dependence, and is becoming widely applied with each passing day. In this paper, the purpose is to determine the early diagnosis and treatment modalities for Diffuse Lamellar Keratitis, and to examine current approaches for the prevention of permanent visual impairment, which may lead to progressive and irreversible corneal and visual damage.
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