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Fuchs' corneal dystrophy

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https://www.readbyqxmd.com/read/28738416/conditionally-immortal-slc4a11-mouse-corneal-endothelial-cell-line-recapitulates-disrupted-glutaminolysis-seen-in-slc4a11-mouse-model
#1
Wenlin Zhang, Diego G Ogando, Edward T Kim, Moon-Jung Choi, Hongde Li, Jason M Tenessen, Joseph A Bonanno
Purpose: To establish conditionally immortal mouse corneal endothelial cell lines with genetically matched Slc4a11+/+ and Slc4a11-/- mice as a model for investigating pathology and therapies for SLC4A11 associated congenital hereditary endothelial dystrophy (CHED) and Fuchs' endothelial corneal dystrophy. Methods: We intercrossed H-2Kb-tsA58 mice (Immortomouse) expressing an IFN-γ dependent and temperature-sensitive mutant of the SV40 large T antigen (tsTAg) with Slc4a11+/+ and Slc4a11-/- C57BL/6 mice...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28731879/peripheral-endothelial-cell-count-is-a-predictor-of-disease-severity-in-advanced-fuchs-endothelial-corneal-dystrophy
#2
Zeba A Syed, Jennifer A Tran, Ula V Jurkunas
PURPOSE: In advanced Fuchs endothelial corneal dystrophy (FECD), central endothelial changes do not correlate with disease severity. The peripheral endothelial cell count (ECC) has not been studied as a marker of FECD severity. The goal of this study was to determine the relationship between the peripheral ECC and known clinical markers of FECD in advanced cases. METHODS: Patients with FECD examined between January 1, 2013, and September 1, 2016, by 1 cornea specialist were identified...
July 19, 2017: Cornea
https://www.readbyqxmd.com/read/28727885/sustained-activation-of-the-unfolded-protein-response-induces-cell-death-in-fuchs-endothelial-corneal-dystrophy
#3
Naoki Okumura, Miu Kitahara, Hirokazu Okuda, Keisuke Hashimoto, Emi Ueda, Makiko Nakahara, Shigeru Kinoshita, Robert D Young, Andrew J Quantock, Theofilos Tourtas, Ursula Schlötzer-Schrehardt, Friedrich Kruse, Noriko Koizumi
Purpose: The unfolded protein response (UPR) is believed to play a role in the pathogenesis of Fuchs' endothelial corneal dystrophy (FECD). The purpose of this study was to investigate whether unfolded proteins accumulate in the corneal endothelium in FECD and if they are involved in triggering cell death. Methods: Descemet's membranes with corneal endothelial cells (CECs) were obtained during keratoplasty, and expression of aggresomes, type 1 collagen, fibronectin, and agrin was evaluated...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28726551/extracellular-matrix-and-integrin-expression-profiles-in-fuchs-endothelial-corneal-dystrophy-cells-and-tissue-model
#4
Benjamin Goyer, Mathieu Thériault, Sébastien P Gendron, Isabelle Brunette, Patrick J Rochette, Stéphanie Proulx
Primary corneal endothelial cell cultures and 3D engineered tissue models were used to study the aberrant deposition of extracellular matrix (ECM) in a vision impairing pathology known as Fuchs endothelial corneal dystrophy (FECD). Corneal endothelial cells (CEC) were isolated from excised Descemet membranes of patients with end-stage FECD. CEC isolated from healthy corneas served as controls. Microarray gene profiling was performed on post-confluent cultures of healthy and FECD cells. Protein expression analyses were conducted on tissue models that were engineered by seeding an endothelium on previously devitalized human stromal carriers...
July 20, 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/28726355/combined-cataract-dsek-dmek-changing-expectations
#5
Francis W Price, Marianne O Price
Endothelial keratoplasty (EK) has revolutionized corneal transplant surgery by providing rapid visual recovery and improved visual outcomes. In parts of the world with Fuchs endothelial dystrophy, many patients may present with both cataracts and corneal degeneration requiring surgery. Other forms of endothelial decompensation may also present with cataracts. The staging or combination of transplant surgery and cataract surgery depend on both the abilities of the surgeon and the surgical techniques being considered...
July 18, 2017: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/28697678/ek-dlek-dsek-dmek-new-frontier-in-cornea-surgery
#6
Marianne O Price, Pankaj Gupta, Jonathan Lass, Francis W Price
Endothelial keratoplasty (EK) has revolutionized treatment of corneal endothelial dysfunction. Compared with penetrating keratoplasty (PK), EK provides faster and more reliable visual rehabilitation while maintaining the eye's structural integrity. The number of EK procedures is growing annually and surpassed PK in the United States in 2012. The most widely used iteration, Descemet stripping endothelial keratoplasty (DSEK), implants healthy donor endothelium, Descemet membrane, and posterior stroma. Descemet membrane endothelial keratoplasty (DMEK) eliminates the donor stromal layer...
July 11, 2017: Annual Review of Vision Science
https://www.readbyqxmd.com/read/28642546/slc4a11-depletion-impairs-nrf2-mediated-antioxidant-signaling-and-increases-reactive-oxygen-species-in-human-corneal-endothelial-cells-during-oxidative-stress
#7
Sanjukta Guha, Sunita Chaurasia, Charanya Ramachandran, Sanhita Roy
Corneal endothelial dystrophy is a progressive disease with gradual loss of vision and characterized by degeneration and dysfunction of corneal endothelial cells. Mutations in SLC4A11, a Na(+) dependent OH(-) transporter, cause congenital hereditary endothelial dystrophy (CHED) and Fuchs' endothelial corneal dystrophy (FECD), the two most common forms of endothelial degeneration. Along with genetic factors, oxidative stress plays a role in pathogenesis of several corneal diseases. In this study we looked into the role of SLC4A11 in antioxidant stress response in human corneal endothelial cells (HCEnC)...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28608272/ctg18-1-repeat-expansion-may-reduce-tcf4-gene-expression-in-corneal-endothelial-cells-of-german-patients-with-fuchs-dystrophy
#8
Sabine Foja, Mirjam Luther, Katrin Hoffmann, Andreas Rupprecht, Claudia Gruenauer-Kloevekorn
PURPOSE: It was the aim of this investigation to elucidate the functional effects of CTG18.1 trinucleotide repeat expansion and the polymorphism rs613872 in the transcription factor 4 (TCF4) in corneas of patients affected by Fuchs' endothelial corneal dystrophy (FECD). METHODS: Sixty-one unrelated German patients with FECD and 113 unaffected controls were investigated and genotyped for the CTG18.1 locus by triplet primed PCR (TP-PCR) and the rs613872 polymorphism via Sanger sequencing and by employing genomic DNA from peripheral blood leucocytes...
June 12, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/28600303/stability-of-visual-outcome-between-2-and-5-years-following-corneal-transplantation-in-the-uk
#9
Sing-Pey Chow, Cathy L Hopkinson, Derek M Tole, Mark N Jones, Stuart D Cook, W John Armitage
BACKGROUND AND AIMS: Many studies of corneal transplantation focus on graft failure or rejection as endpoints, or report visual outcomes at one postoperative time point. We aimed to study the stability of visual outcomes between 2 and 5 years following corneal transplantation. METHODS: All patients with keratoconus (868) or Fuchs endothelial dystrophy (FED) (569) receiving their first corneal transplant for visual purposes in the UK between January 2003 and December 2009 were included...
June 9, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28597204/-descemetectomy-an-alternative-to-transplantation
#10
REVIEW
E L Bramann, K Schröder, S Schrader, G Geerling
Due to rising demand worldwide it is becoming increasingly more difficult to satisfy the needs for human corneal transplants. At this point transplantation is the only possible cure for endothelial diseases, such as Fuchs endothelial dystrophy. In this article we present descemetectomy, a circumscribed removal of Descemet's membrane (DMx) without subsequent transplantation, as new possible treatment option on the basis of a clinical case.
July 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/28594697/comparison-of-femtosecond-laser-enabled-descemetorhexis-and-manual-descemetorhexis-in-descemet-membrane-endothelial-keratoplasty
#11
Adi Einan-Lifshitz, Nir Sorkin, Tanguy Boutin, Mahmood Showail, Armand Borovik, Murad Alobthani, Clara C Chan, David S Rootman
PURPOSE: To introduce a novel method to perform descemetorhexis in Descemet membrane endothelial keratoplasty (DMEK) using the femtosecond laser and to compare it with Descemet membrane endothelial keratoplasty performed with manual descemetorhexis (M-DMEK). METHODS: A retrospective medical chart review of 2 groups of patients who underwent DMEK surgery combined with cataract surgery secondary to Fuchs corneal endothelial dystrophy and cataract: 17 patients underwent femtosecond laser-enabled descemetorhexis Descemet membrane endothelial keratoplasty (FE-DMEK) and 89 patients underwent DMEK surgery with M-DMEK...
July 2017: Cornea
https://www.readbyqxmd.com/read/28549842/mitochondrial-dysfunction-and-oxidative-stress-in-corneal-disease
#12
Neeru A Vallabh, Vito Romano, Colin E Willoughby
The cornea is the anterior transparent surface and the main refracting structure of the eye. Mitochondrial dysfunction and oxidative stress are implicated in the pathogenesis of inherited (e.g. Kearns Sayre Syndrome) and acquired corneal diseases (e.g. keratoconus and Fuchs endothelial corneal dystrophy). Both antioxidants and reactive oxygen species are found in the healthy cornea. There is increasing evidence of imbalance in the oxidative balance and mitochondrial function in the cornea in disease states...
May 23, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28525563/omics-biomarkers-in-ophthalmology
#13
REVIEW
Susette Lauwen, Eiko K de Jong, Dirk J Lefeber, Anneke I den Hollander
"Omics" refers to high-throughput analyses of genes, proteins, or metabolites in a biological system, and is increasingly used for ophthalmic research. These system-based approaches can unravel disease-related processes and are valuable for biomarker discovery. Furthermore, potential therapeutic targets can be identified based on omics results, and targeted follow-up experiments can be designed to gain molecular understanding of the disease and to test new therapies. Here, we review the application of omics techniques in eye diseases, focusing on age-related macular degeneration (AMD), diabetic retinopathy (DR), retinal detachment (RD), myopia, glaucoma, Fuchs' corneal dystrophy (FCD), cataract, keratoconus, and dry eyes...
May 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28520609/fuchs-endothelial-corneal-dystrophy-in-a-child
#14
Sunita Chaurasia, Muralidhar Ramappa
No abstract text is available yet for this article.
May 16, 2017: Cornea
https://www.readbyqxmd.com/read/28489792/eye-bank-prepared-versus-surgeon-cut-endothelial-graft-tissue-for-descemet-membrane-endothelial-keratoplasty-an-observational-study
#15
COMPARATIVE STUDY
Marie Regnier, Céline Auxenfans, Delphine Maucort-Boulch, Anne-Sophie Marty, Odile Damour, Carole Burillon, Viridiana Kocaba
The purpose of this article is to examine outcomes of Descemet membrane endothelial keratoplasty (DMEK) performed with cornea bank (CB) prestripped tissue and surgeon stripped tissue (SST).This retrospective study examined subjects who underwent DMEK with CB or surgeon prepared tissue for Fuchs endothelial corneal dystrophy. Best-corrected visual acuity (BCVA), corneal thickness, endothelial cell count (ECC), and complications were examined before and throughout a 6-month postoperative period.Eleven CB and 22 SST subjects were included...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28486726/management-of-interface-fluid-syndrome-after-lasik-by-descemet-membrane-endothelial-keratoplasty-in-a-patient-with-fuchs-corneal-endothelial-dystrophy
#16
Mehdi Shajari, Parviz Rafiezadeh, Ivana Pavlovic, Kate Barbara Kubiak, Thomas Kohnen, Ingo Schmack
PURPOSE: To report a case of interface fluid syndrome after LASIK in a patient with Fuchs' corneal endothelial dystrophy treated with Descemet membrane endothelial keratoplasty (DMEK). METHODS: Case report. RESULTS: A 74-year-old patient presented with interface fluid syndrome and was treated with DMEK. Its complications in the form of partial graft detachment and cystoid macular edema were successfully managed by rebubbling and topical treatment, respectively...
May 1, 2017: Journal of Refractive Surgery
https://www.readbyqxmd.com/read/28481834/immunohistochemical-profiling-of-corneas-with-fuchs-endothelial-corneal-dystrophy
#17
An-Katrien De Roo, Thomas Janssens, Beatrijs Foets, Joost J van den Oord
PURPOSE: Fuchs endothelial corneal dystrophy (FECD) is the leading indication for endothelial keratoplasty. Further insight into its pathophysiology is needed to develop alternative therapies. METHODS: Sixteen genes from a previous microarray expression experiment (FECD vs. normal) were validated using immunohistochemistry on paraffin-embedded corneas (n = 6 FECD, n = 6 normal). The results were quantified manually and semiautomatically. RESULTS: A higher percentage of corneal endothelial cells stained for alpha-smooth muscle actin (αSMA), cytokeratin 7, and superoxide dismutase 3 in FECD versus normal [odds ratios (ORs) of 60...
July 2017: Cornea
https://www.readbyqxmd.com/read/28476048/descemetorhexis-without-grafting-for-fuchs-endothelial-dystrophy-supplementation-with-topical-ripasudil
#18
Gregory Moloney, Constantinos Petsoglou, Matthew Ball, Yves Kerdraon, Roland Höllhumer, Natasha Spiteri, Simone Beheregaray, Judith Hampson, Mario DʼSouza, Raj N Devasahayam
PURPOSE: To report the safety and efficacy of descemetorhexis without grafting as a primary intervention in Fuchs dystrophy, and the use of a ROCK inhibitor, ripasudil as a salvage agent in failing cases. METHODS: Twelve eyes of 11 patients underwent central descemetorhexis not exceeding 4 mm. All had Fuchs dystrophy-producing visual symptoms, requesting intervention. Exclusion criteria were a peripheral endothelial cell count <1000 and central edema. Corneal clearance and visual parameters were recorded monthly until corneal clearance was observed, then at intervals of 6 months...
June 2017: Cornea
https://www.readbyqxmd.com/read/28437275/descemet-membrane-endothelial-keratoplasty-after-failed-descemet-stripping-without-endothelial-keratoplasty
#19
Rohini Rao, Durga S Borkar, Kathryn A Colby, Peter B Veldman
PURPOSE: To describe the clinical course, surgical experience, and postoperative outcomes of 3 patients with Fuchs endothelial dystrophy who underwent Descemet membrane endothelial keratoplasty (DMEK) after failed Descemet stripping without endothelial keratoplasty. METHODS: Three patients who underwent DMEK for management of persistent corneal edema after deliberate Descemet stripping in the setting of Fuchs endothelial dystrophy were identified. Patients were examined at day 1, week 1, and months 1, 3, and 6 after DMEK...
July 2017: Cornea
https://www.readbyqxmd.com/read/28410548/two-year-clinical-outcome-of-500-consecutive-cases-undergoing-descemet-membrane-endothelial-keratoplasty
#20
Jorge Peraza-Nieves, Lamis Baydoun, Isabel Dapena, Abbas Ilyas, Laurence E Frank, Salvatore Luceri, Lisanne Ham, Silke Oellerich, Gerrit R J Melles
PURPOSE: To evaluate the clinical outcome of 500 consecutive cases up to 2 years after Descemet membrane endothelial keratoplasty (DMEK) and to assess which parameters may have influenced the clinical outcome. METHODS: From a group of 500 eyes (393 patients), which underwent DMEK for Fuchs endothelial corneal dystrophy (FECD), bullous keratopathy, failed corneal transplants and other indications, clinical outcomes [best-corrected visual acuity (BCVA), central endothelial cell density (ECD), and central corneal thickness] were evaluated before, and at 6, 12, and 24 months after DMEK and postoperative complications were documented...
June 2017: Cornea
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