Reduced Electroretinogram Responses in Morphologically Normal Retina in Patients with Primary Hyperoxaluria Type 1.

Purpose: To describe ocular findings in individuals with primary hyperoxaluria type 1 (PH1), focusing on the correlations between retinal anatomy and retinal function. To characterize the retinal alterations that occur at different disease stages by evaluating individuals with diverse degrees of renal impairment associated with PH1.

Design: A cross-sectional study.

Induction of retinopathy by fibrillar oxalate assemblies.

The formation of metabolite fibrillar assemblies represents a paradigm shift in the study of human metabolic disorders. Yet, direct clinical relevance has been attributed only to metabolite crystals. A notable example for metabolite crystallization is calcium oxalate crystals observed in various diseases, including primary hyperoxaluria.

ISCEV extended protocol for the S-cone ERG.

The International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum procedure for testing generalized retinal function but encourages more extensive testing. This extended protocol describes a method of assessing the function of the short-wavelength-sensitive cone (S-cone) retinal pathway, using a short-wavelength flash superimposed on a background that saturates the rods and adapts the L/M-cones to elicit a response, known as the S-cone ERG. Stimulus parameters such as the strength and luminance of the flash and background, respectively, and their spectral and temporal characteristics are specified.

PRCD is concentrated at the base of photoreceptor outer segments and is involved in outer segment disc formation.

Mutations of the photoreceptor disc component (PRCD) gene are associated with rod-cone degeneration in both dogs and humans. Prcd is expressed in the mouse eye as early as embryonic day 14. In the adult mouse retina, PRCD is expressed in the outer segments of both rod and cone photoreceptors.

Verifying complaints of difficulties in night vision using electroretinography and dark adaptation tests.

Purpose: To determine the electroretinographical and psychophysical parameters that can help to verify patients' complaints of reduced night vision.

Methods: We tested 275 consecutive patients with normal appearing fundi, complaining of visual difficulties at night, using flash electroretinography (ERG) and dark adaptation (DA) test. Two ERG parameters were used to assess a scotopic retinal function: the amplitude of the response to dim blue flash (the rod response) and the b-wave ratio (measured/expected).

[THE ISRAELI INHERITED RETINAL DISEASES CONSORTIUM (IIRDC)- CLINICAL-GENETIC MAPPING AND FUTURE PERSPECTIVES].

Introduction: The sense of vision is highly important for humans and its loss markedly affects function and quality of life. Many inherited retinal diseases (IRDs) cause visual loss due to dysfunction or progressive degeneration of photoreceptor cells. These diseases show clinical and genetic heterogeneity.

Retinal Toxicity of Intravitreal Injection of Ziv-Aflibercept in Albino Rabbits.

Purpose: To evaluate retinal toxicity of ziv-aflibercept, a drug that had been approved for use for patients with colon cancer.

Methods: Twenty-two albino rabbits were injected intravitreally with 0.1 mL of ziv-aflibercept solution into the experimental eye and 0.

Intravitreal Trimethoprim and Sulfamethoxazole Toxicity to the Retina of Albino Rabbits.

Purpose: To evaluate retinal toxicity of intravitreal trimethoprim-sulfamethoxazole (TMP-SMX) in an albino rabbit model.

Methods: Albino rabbits ( = 10) were treated in the right eye with the maximum intravitreal dose of TMP-SMX mixture (1600 μg/8000 μg /0.1 mL), while 0.

ISCEV extended protocol for the dark-adapted red flash ERG.

The International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum procedure, but encourages more extensive testing. This ISCEV extended protocol describes an extension to the ERG standard, namely the dark-adapted (DA) red flash ERG. The DA red flash ERG can be incorporated conveniently within the ISCEV standard ERG protocol after a minimum of 20-min DA and recorded after the DA 0.

ATF3 Regulates the Expression of AChE During Stress.

Acetylcholinesterase (AChE) expresses in non-cholinergic cells, but its role(s) there remain unknown. We have previously attributed a pro-apoptotic role for AChE in stressed retinal photoreceptors, though by unknown mechanism. Here, we examined its promoter only to find that it includes a binding sequence for the activating transcription factor 3 (ATF3); a prototypical mediator of apoptosis.

Infliximab exerts a dose-dependent effect on retinal safety in the albino rabbit.

Purpose: To assess the retinal toxicity of an intravitreal injection of infliximab, a monoclonal antibody to tumor necrosis factor α, in a rabbit model.

Materials And Methods: Two groups of adult albino rabbits (n = 5) received intravitreal injections of infliximab (0.1 ml) in the study eye and balanced salt solution (BSS, 0.

The role of nitric oxide in spectral information processing in the distal turtle retina.

Chromaticity type horizontal cells (C-type HCs) are the first retinal neurons exhibiting spectral information processing in cold-blooded vertebrates. The simple input of hyperpolarizing responses of cone photoreceptors is transformed in the C-type HCs into spectral opponent output. Nitric oxide (NO), a known background neuromodulator in the distal retina, was tested here for its effects upon spectral information processing by C-type HCs in the retina of turtle.

Safety of intravitreal clindamycin in albino rabbit eyes.

Purpose: To study the potential toxic effects of intravitreal clindamycin on the retina of albino rabbits, by assessing functional and morphological retinal changes.

Methods: Eight albino rabbits were included in the study. In each rabbit, 1 mg/0.

Light Modulates Ocular Complications in an Albino Rat Model of Type 1 Diabetes Mellitus.

Purpose: The purpose of the study was to assess potential interactions of light exposure and hyperglycemia upon ocular complications in diabetic rats.

Methods: Streptozotocin-induced (STZ-induced) diabetic rats ( = 39) and non-diabetic rats ( = 9) were distributed into eight groups according to the irradiance and color of the light phase during the 12/12-hour light/dark regime. Follow-up lasted 90 days and included assessment of cataract development and electroretinogram (ERG) recordings.

Homozygosity for a Recessive Loss-of-Function Mutation of the NRL Gene Is Associated With a Variant of Enhanced S-Cone Syndrome.

Purpose: To investigate the genetic basis for severe visual complaints by Bukharan Jewish patients with oculopharyngeal muscular dystrophy (OPMD).

Methods: Polymerase chain reaction amplification and direct sequencing were used to test for NRL, PABPN1, and NR2E3 mutations. Complete ophthalmic examination included best-corrected visual acuity, biomicroscopic examination, optical coherence tomography, and fundus autofluorescence.

An intronic deletion in the PROM1 gene leads to autosomal recessive cone-rod dystrophy.

Purpose: To investigate the genetic basis for autosomal recessive cone-rod dystrophy (CRD) in a consanguineous Israeli Jewish family.

Methods: Patients underwent a detailed ophthalmic evaluation, including eye examination, visual field testing, optical coherence tomography (OCT), and electrophysiological tests, electroretinography (ERG) and visual evoked potential (VEP). Genome-wide homozygosity mapping using a single nucleotide polymorphism (SNP) array was performed to identify homozygous regions shared among two of the affected individuals.

Müller cells separate between wavelengths to improve day vision with minimal effect upon night vision.

Vision starts with the absorption of light by the retinal photoreceptors-cones and rods. However, due to the 'inverted' structure of the retina, the incident light must propagate through reflecting and scattering cellular layers before reaching the photoreceptors. It has been recently suggested that Müller cells function as optical fibres in the retina, transferring light illuminating the retinal surface onto the cone photoreceptors.

The effects of excitatory amino acids and their transporters on function and structure of the distal retina in albino rabbits.

Purpose: To study the physiological and pathological roles of excitatory amino acid transporters in the distal retina of albino rabbits.

Methods: Albino rabbits were injected intravitreally in one eye with different doses of L- or D-isomers of glutamate or aspartate, with mixtures of L-glutamate and antagonists to glutamate receptors or with inhibitors of glutamate transporters. The other eye was injected with saline, and served as a control.

Retinal toxicity of intravitreal rituximab in albino rabbits.

Purpose: To evaluate retinal toxicity of intravitreal rituximab.

Methods: Twelve albino rabbits were included in the study. 1 mg/0.