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Electroretinogram

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https://www.readbyqxmd.com/read/28929832/cngb3-mutations-cause-severe-rod-dysfunction
#1
J Maguire, M McKibbin, K Khan, S Kohl, M Ali, D McKeefry
PURPOSE: Congenital achromatopsia or rod monochromatism is a rare autosomal recessive condition defined by a severe loss of cone photoreceptor function in which rods purportedly retain normal or near-to-normal function. This report describes the results of electroretinography in two siblings with CNGB3-associated achromatopsia. METHODS: Full field light- and dark-adapted electroretinograms (ERGs) were recorded using standard protocols detailed by the International Society for Clinical Electrophysiology of Vision (ISCEV)...
September 20, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28926202/retinal-dysfunction-characterizes-subtypes-of-dominant-optic-atrophy
#2
Maria Lucia Cascavilla, Vincenzo Parisi, Giacinto Triolo, Lucia Ziccardi, Enrico Borrelli, Antonio Di Renzo, Nicole Balducci, Costanza Lamperti, Stefania Bianchi Marzoli, Fatima Darvizeh, Alfredo A Sadun, Valerio Carelli, Francesco Bandello, Piero Barboni
PURPOSE: To assess preganglionic retinal function using multifocal electroretinogram (mfERG) in patients affected by dominant optic atrophy (DOA) stratified by OPA1 gene mutation. METHODS: Multifocal electroretinogram (mfERG) was recorded in 18 DOA patients (DOA group, 35 eyes) and 25 age-matched healthy subjects (control group, 25 eyes). Patients were stratified in two groups based on gene mutation: missense mutation (DOA-M group, 11 eyes) and mutation causing haploinsufficiency (DOA-H group, 24 eyes)...
September 19, 2017: Acta Ophthalmologica
https://www.readbyqxmd.com/read/28917993/nonsedated-handheld-electroretinogram-as-a-screening-test-of-retinal-dysfunction-in-pediatric-patients-with-nystagmus
#3
Sara F Grace, Byron L Lam, William J Feuer, Carla J Osigian, Kara M Cavuoto, Hilda Capo
PURPOSE: To assess the feasibility, sensitivity, and specificity of nonsedated handheld cone flicker electroretinogram (ERG) as a screening tool to detect retinal dysfunction in children with nystagmus. METHODS: Pediatric patients at a tertiary referral center from December 2015 to July 2016 were enrolled and placed into three age-matched groups: normal, nystagmus with a retinal dystrophy, and nystagmus without a retinal dystrophy. Unsedated 30 Hz cone flicker ERG responses were obtained using a handheld device (RETeval) from both eyes of each patient using skin electrode sensors after pupillary dilation...
September 13, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28912967/correlation-of-macular-focal-electroretinogram-with-ellipsoid-zone-extension-in-stargardt-disease
#4
Edoardo Abed, Giorgio Placidi, Luigi Calandriello, Marco Piccardi, Francesca Campagna, Matteo Bertelli, Angelo Maria Minnella, Maria Cristina Savastano, Benedetto Falsini
Stargardt disease (STGD1) is the most common cause of inherited juvenile macular degeneration. This disease is characterized by a progressive accumulation of lipofuscin in the outer retina and subsequent loss of photoreceptors and retinal pigment epithelium. The aim of this study was to evaluate the relationship between cone photoreceptor function and structure in STGD1. Macular function was assessed by visual acuity measurement and focal electroretinogram (FERG) recording while spectral domain optical coherence tomography (SD-OCT) imaging was performed to evaluate the integrity of photoreceptors...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/28912962/gene-panel-sequencing-in-brazilian-patients-with-retinitis-pigmentosa
#5
Kárita Antunes Costa, Mariana Vallim Salles, Chris Whitebirch, John Chiang, Juliana Maria Ferraz Sallum
BACKGROUND: Retinal dystrophies constitute a group of diseases characterized by clinical variability and pronounced genetic heterogeneity. Retinitis pigmentosa is the most common subtype of hereditary retinal dystrophy and is characterized by a progressive loss of peripheral field vision (Tunnel Vision), eventual loss of central vision, and progressive night blindness. The characteristics of the fundus changes include bone-spicule formations, attenuated blood vessels, reduced and/or abnormal electroretinograms, changes in structure imaged by optical coherence tomography, and subjective changes in visual function...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/28906472/therapeutic-effect-of-cilostazol-ophthalmic-nanodispersions-on-retinal-dysfunction-in-streptozotocin-induced-diabetic-rats
#6
Noriaki Nagai, Saori Deguchi, Hiroko Otake, Noriko Hiramatsu, Naoki Yamamoto
We previously prepared ophthalmic formulations containing cilostazol (CLZ) nanoparticles by bead mill methods (CLZnano), and found that instillation of CLZnano into rat eyes supplies CLZ into the retina. In this study, we investigated changes in the electroretinograms (ERG) of streptozotocin-induced diabetic rats (STZ rats), a model of diabetes mellitus. In addition, we demonstrated that dispersions containing CLZ nanoparticles attenuate changes in the ERG of STZ rats. The instillation of CLZnano had no effect on body weight or plasma glucose and insulin levels...
September 14, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28902720/evaluation-of-the-macula-prior-to-cataract-surgery
#7
Marta McKeague, Priya Sharma, Allen C Ho
PURPOSE OF REVIEW: To describe recent evidence regarding methods of evaluation of retinal structure and function prior to cataract surgery. RECENT FINDINGS: Studies in patients with cataract but no clinically detectable retinal disease have shown that routine use of optical coherence tomography (OCT) prior to cataract surgery can detect subtle macular disease, which may alter the course of treatment or lead to modification of consent. The routine use of OCT has been especially useful in patients being considered for advanced-technology intraocular lenses (IOLs) as subtle macular disease can be a contraindication to the use of these lenses...
September 11, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28900746/electrophysiological-features-and-multimodal-imaging-in-ritonavir-related-maculopathy
#8
Céline Faure, Michel Paques, Isabelle Audo
PURPOSE: The purpose of this study is to report a case of ritonavir-related retinal toxicity followed over a year. Electrophysiological features and multimodal imaging, including adaptive optics, are provided and discussed. METHODS: Electrophysiological recordings and multimodal imaging were performed and repeated over 1 year. RESULTS: Fundus examination revealed crystalline maculopathy in conjunction with pigment disruption. Spectral domain optical coherence tomography displayed thinning of the macula without cysts...
September 12, 2017: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/28894305/electroretinography-reveals-difference-in-cone-function-between-syndromic-and-nonsyndromic-ush2a-patients
#9
Jesse D Sengillo, Thiago Cabral, Kaspar Schuerch, Jimmy Duong, Winston Lee, Katherine Boudreault, Yu Xu, Sally Justus, Janet R Sparrow, Vinit B Mahajan, Stephen H Tsang
Usher syndrome is an inherited and irreversible disease that manifests as retinitis pigmentosa (RP) and bilateral neurosensory hearing loss. Mutations in Usherin 2A (USH2A) are not only a frequent cause of Usher syndrome, but also nonsyndromic RP. Although gene- and cell-based therapies are on the horizon for RP and Usher syndrome, studies characterizing natural disease are lacking. In this retrospective analysis, retinal function of USH2A patients was quantified with electroretinography. Both groups had markedly reduced rod and cone responses, but nonsyndromic USH2A patients had 30 Hz-flicker electroretinogram amplitudes that were significantly higher than syndromic patients, suggesting superior residual cone function...
September 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28885867/clinical-characteristics-of-recessive-retinal-degeneration-due-to-mutations-in-the-cdhr1-gene-and-a-review-of-the-literature
#10
A P Bessette, M J DeBenedictis, E I Traboulsi
BACKGROUND: The clinical phenotype of patients presenting with autosomal recessive CDHR1-related retinopathy has not been well described. MATERIALS AND METHODS: This is a retrospective case series of patients presenting to a single institution. Clinical data, including age, visual acuity, dilated fundus exam, fundus photos, fundus autofluorescence (FAF), optical coherence tomography, full-field electroretinograms (ERGs), and results of genetic testing, were collected...
September 8, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28878288/abnormal-electroretinogram-after-kir7-1-channel-suppression-suggests-role-in-retinal-electrophysiology
#11
Pawan K Shahi, Xinling Liu, Bryce Aul, Andrea Moyer, Akshita Pattnaik, Jerod Denton, De-Ann M Pillers, Bikash R Pattnaik
The KCNJ13 gene encodes the inwardly rectifying potassium channel, Kir7.1. Mutations in this gene cause childhood blindness, in which the a- and b-wave responses of electroretinogram (ERG) are abolished. The ERG a-wave is the light-induced hyperpolarization of retinal photoreceptors, and the b-wave is the depolarization of ON-bipolar cells. The Kir7.1 channel is localized to the apical aspects of retinal pigment epithelium (RPE) cells and contributes to a delayed c-wave response. We sought to understand why a defect in an RPE ion-channel result in abnormal electrophysiology at the level of the retinal neurons...
September 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28867866/comparative-investigation-of-stimulus-evoked-rod-outer-segment-movement-and-retinal-electrophysiological-activity
#12
Yiming Lu, Benquan Wang, Xincheng Yao
Transient retinal phototropism (TRP) has been observed in rod photoreceptors activated by oblique visible light flashes. Time-lapse confocal microscopy and optical coherence tomography (OCT) revealed rod outer segment (ROS) movements as the physical source of TRP. However, the physiological source of TRP is still not well understood. In this study, concurrent TRP and electroretinogram (ERG) measurements disclosed a remarkably earlier onset time of the ROS movements (≤10 ms) than that (∼38 ms) of the ERG a-wave...
January 28, 2017: Proceedings of SPIE
https://www.readbyqxmd.com/read/28867704/evaluation-of-retinal-function-in-streptozotocin-induced-diabetic-rats-by-using-the-electroretinography-and-immunohistochemistry-methods
#13
Noriko Hiramatsu, Saori Deguchi, Chiaki Yoshioka, Hiroko Otake, Naoki Yamamoto, Noriaki Nagai
 Streptozotocin-induced diabetic rat (STZ rat) was used in many studies for the diabetic mellitus. In this study, we demonstrated whether the electroretinograms (ERG) was changed in the retina of STZ rats. In addition, we investigated the histopathological alteration in the retina of STZ rats by using the immunological method. The 100 mg/kg of STZ was injected continuously for 2 d (100 mg/kg×2). The insulin level was decreased, and the glucose level was enhanced 14 d after the injection of STZ. Moreover, the levels of a-wave, b-wave and OP amplitude were decreased in the rat at 14 d after the injection of STZ...
2017: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://www.readbyqxmd.com/read/28861345/possible-role-of-sialylation-of-retinal-protein-glycans-in-the-regulation-of-electroretinogram-response-in-mice
#14
Satpal Ahuja
AIM: To evaluate if the nature, degree and extent of Siaα2-3-/Siaα2-6-sialylation of retinal protein glycans plays a possible role in the development and regulation of electroretinogram response (ERG) in mice. METHODS: Proteins extracted, from retinae of postnatal day 2 (PN2), PN7, and PN14 wild type (wt) and retinal degeneration 1 (rd1) mice were quantified, labeled and used for lectin-microarray profiling with immobilized lectins which recognize a wide range of N-/O-glycans...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28845542/cofactors-associated-with-sudden-acquired-retinal-degeneration-syndrome-151-dogs-within-a-reference-population
#15
Candace R Auten, Sara M Thomasy, Philip H Kass, Kathryn L Good, Steven R Hollingsworth, David J Maggs
OBJECTIVE: To determine factors associated with sudden acquired retinal degeneration syndrome (SARDS) diagnosed within one referral population. ANIMALS STUDIED: 151 dogs diagnosed with SARDS. PROCEDURES: Breed, age, sex, and body weight were compared between dogs with electroretinogram-confirmed SARDS and dogs presented to the UC Davis Veterinary Medical Teaching Hospital (UCD-VMTH) from 1991 to 2014. RESULTS: SARDS was diagnosed in 151 dogs, representing 1...
August 27, 2017: Veterinary Ophthalmology
https://www.readbyqxmd.com/read/28841046/electroretinographic-assessment-of-inner-retinal-signaling-in-the-isolated-and-superfused-murine-retina
#16
Walid Albanna, Jan Niklas Lueke, Volha Sjapic, Konstantin Kotliar, Jürgen Hescheler, Hans Clusmann, Sergej Sjapic, Serdar Alpdogan, Toni Schneider, Gerrit Alexander Schubert, Felix Neumaier
PURPOSE: Longer-lasting electroretinographic recordings of the isolated murine retina were initially achieved by modification of a phosphate-buffered nutrient solution originally developed for the bovine retina. During experiments with a more sensitive mouse retina, apparent model-specific limitations were addressed and improvements were analyzed for their contribution to an optimized full electroretinogram (ERG). MATERIAL AND METHODS: Retinas were isolated from dark-adapted mice, transferred to a recording chamber and superfused with different solutions...
August 25, 2017: Current Eye Research
https://www.readbyqxmd.com/read/28839191/loss-of-tmem30a-leads-to-photoreceptor-degeneration
#17
Lin Zhang, Yeming Yang, Shujin Li, Shanshan Zhang, Xiong Zhu, Zhengfu Tai, Mu Yang, Yuqing Liu, Xinzheng Guo, Bo Chen, Zhilin Jiang, Fang Lu, Xianjun Zhu
Phosphatidylserine (PS) is asymmetrically distributed between the outer and inner leaflets of the plasma membrane in eukaryotic cells. PS asymmetry on the plasma membrane depends on the activities of P4-ATPases, and disruption of PS distribution can lead to various disease conditions. Folding and transporting of P4-ATPases to their cellular destination requires the β subunit TMEM30A proteins. However, the in vivo functions of Tmem30a remain unknown. To this end, we generated retinal-specific Tmem30a-knockout mice to investigate its roles in vivo for the first time...
August 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28831547/developmental-visual-deprivation-long-term-effects-on-human-cone-driven-retinal-function
#18
Paolo Esposito Veneruso, Lucia Ziccardi, Giulia Magli, Vincenzo Parisi, Benedetto Falsini, Adriano Magli
PURPOSE: To assess whether infantile visual deprivation induced by developmental cataract may influence the cone-driven retinal function in humans. METHODS: A total of 14 patients with history of bilateral developmental cataract (DC), who had undergone uncomplicated cataract extraction surgery and intraocular lens implant, and 14 healthy subjects (HS) were enrolled. All patients underwent complete ophthalmological and orthoptic evaluations and best-corrected visual acuity measurement...
August 22, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/28815346/unilateral-cancer-associated-retinopathy-diagnosis-serology-and-treatment
#19
Dimitri Roels, Shinji Ueno, Cornelia D Talianu, Dafina Draganova, Mineo Kondo, Bart P Leroy
PURPOSE: To report a case of unilateral cancer-associated retinopathy (CAR) with clinical, serological and electroretinogram (ERG) normalization after aggressive cancer treatment combined with steroids and rituximab. METHODS: Work-up included extensive clinical and electrophysiological testing. Also, serological work-up for antiretinal antibodies and oncological screening was organized. RESULTS: A 45-year-old female presented with progressive photopsias, photophobia and relative central scotoma in the right eye since 6 weeks prior...
August 16, 2017: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/28813575/subclinical-decrease-in-central-inner-retinal-activity-is-associated-with-myopia-development-in-children
#20
Serena Zhe-Chuang Li, Wing-Yan Yu, Kai-Yip Choi, Christie Hang-I Lam, Yamunadevi Lakshmanan, Francisca Siu-Yin Wong, Henry Ho-Lung Chan
Purpose: To investigate the characteristics of retinal electrophysiological activity in relation to early myopia development in children. Methods: Fifty-six children aged 6 to 9 years with emmetropic refractive error (defined as ≥ -0.5 diopter [D] and ≤ +0.5 D) were recruited. Cycloplegic refraction, axial length, and global flash multifocal electroretinogram (MOFO mfERG) at 49% and 96% contrast levels were recorded in all children at their first visit. The refraction and axial length measurements were repeated after 1 year...
August 1, 2017: Investigative Ophthalmology & Visual Science
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