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Fuchs' corneal dystrophy

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https://www.readbyqxmd.com/read/28886202/fuchs-endothelial-corneal-dystrophy-and-rna-foci-in-patients-with-myotonic-dystrophy
#1
V Vinod Mootha, Brock Hansen, Ziye Rong, Pradeep P Mammen, Zhengyang Zhou, Chao Xing, Xin Gong
Purpose: The most common cause of Fuchs' endothelial corneal dystrophy (FECD) is an intronic CTG repeat expansion in TCF4. Expanded CUG repeat RNA colocalize with splicing factor, muscleblind-like 1 (MBNL1), in nuclear foci in endothelium as a molecular hallmark. Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder caused by a CTG repeat expansion in the 3'-untranslated region (UTR) of DMPK. In this study, we examine for RNA-MBNL1 foci in endothelial cells of FECD subjects with DM1, test the hypothesis that DM1 patients are at risk for FECD, and determine prevalence of TCF4 and DMPK expansions in a FECD cohort...
September 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28835226/intraocular-lens-power-calculation-using-standard-formulas-and-ray-tracing-after-dmek-in-patients-with-fuchs-endothelial-dystrophy
#2
Maged Alnawaiseh, Lars Zumhagen, André Rosentreter, Nicole Eter
BACKGROUND: The study presented here aims to optimize the accuracy of intraocular lens (IOL) power calculations in patients after DMEK by evaluation of the impact of the altered anterior/posterior corneal curvature relationship. METHODS: Scheimpflug-based Oculus Pentacam imaging was performed after DMEK surgery for Fuchs endothelial dystrophy. The IOL power was calculated for all patients by ray tracing, aiming for postoperative emmetropia. We also performed the IOL calculation using four third-generation formulas (SRK-T, Hoffer-Q, Holladay-1 and Haigis)...
August 23, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28832669/tgc-repeat-expansion-in-the-tcf4-gene-increases-the-risk-of-fuchs-endothelial-corneal-dystrophy-in-australian-cases
#3
Abraham Kuot, Alex W Hewitt, Grant R Snibson, Emmanuelle Souzeau, Richard Mills, Jamie E Craig, Kathryn P Burdon, Shiwani Sharma
Fuchs' endothelial corneal dystrophy (FECD) is a progressive, vision impairing disease. Common single nucleotide polymorphisms (SNPs) and a trinucleotide repeat polymorphism, thymine-guanine-cytosine (TGC), in the TCF4 gene have been associated with the risk of FECD in some populations. We previously reported association of SNPs in TCF4 with FECD risk in the Australian population. The aim of this study was to determine whether TGC repeat polymorphism in TCF4 is associated with FECD in the Australian population...
2017: PloS One
https://www.readbyqxmd.com/read/28782089/the-role-of-lipids-in-corneal-diseases-and-dystrophies-a-systematic-review
#4
REVIEW
Tyler G Rowsey, Dimitrios Karamichos
Corneal diseases are an extensive cause of blindness worldwide and continue to persist as a challenging public health concern. Recently, various lipid-based therapies have been advocated for the modulation of corneal diseases; however, the number of studies is still very limited. Here we focus on developments and challenges on lipid-based therapies for dry eye disease, diabetic neuropathy, and Fuchs' endothelial corneal dystrophy. All three diseases are highly prevalent conditions and involve corneal stress and inflammation...
December 2017: Clinical and Translational Medicine
https://www.readbyqxmd.com/read/28754918/activation-of-tgf-%C3%AE-signaling-induces-cell-death-via-the-unfolded-protein-response-in-fuchs-endothelial-corneal-dystrophy
#5
Naoki Okumura, Keisuke Hashimoto, Miu Kitahara, Hirokazu Okuda, Emi Ueda, Kyoko Watanabe, Makiko Nakahara, Takahiko Sato, Shigeru Kinoshita, Theofilos Tourtas, Ursula Schlötzer-Schrehardt, Friedrich Kruse, Noriko Koizumi
Fuchs endothelial corneal dystrophy (FECD) is a slowly progressive bilateral disease of corneal endothelium in which accumulation of extracellular matrix (ECM) and loss of corneal endothelial cells (CECs) are phenotypic features. The corneal endothelium maintains corneal transparency by regulating water hydration; consequently, corneal endothelial dysfunction causes serious vision loss. The only therapy for corneal haziness due to corneal endothelial diseases, including FECD, is corneal transplantation using donor corneas, and no pharmaceutical treatment is available...
July 28, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28751984/corneal-backscatters-as-an-objective-index-for-assessing-fuchs-endothelial-corneal-dystrophy-a-pilot-study
#6
Hsueh-Yen Chu, Ching-Hsi Hsiao, Phil Yeong-Fong Chen, David Hui-Kang Ma, Chee-Jen Chang, Hsin-Yuan Tan
PURPOSE: To provide an objective, quantitative approach for monitoring Fuchs' endothelial corneal dystrophy (FECD), with Scheimpflug imaging. DESIGN: This is a retrospective case-control pilot study. METHODS: The study group consisted of 53 eyes in 27 patients diagnosed with FECD, with normal subjects paired as control. Main outcome measures were corneal thickness, morphological patterns on densitograms, and indices of corneal density including the average area density (mean AD) and the average ratio of Descemet's membrane density versus area density (DM/AD) in Pentacam Scheimpflug images...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/28751712/activation-of-mitophagy-leads-to-decline-in-mfn2-and-loss-of-mitochondrial-mass-in-fuchs-endothelial-corneal-dystrophy
#7
Anne-Sophie Benischke, Shivakumar Vasanth, Takashi Miyai, Kishore Reddy Katikireddy, Tomas White, Yuming Chen, Adna Halilovic, Marianne Price, Francis Price, Paloma B Liton, Ula V Jurkunas
Human corneal endothelial cells (HCEnCs) are terminally differentiated cells that have limited regenerative potential. The large numbers of mitochondria in HCEnCs are critical for pump and barrier function required for corneal hydration and transparency. Fuchs Endothelial Corneal Dystrophy (FECD) is a highly prevalent late-onset oxidative stress disorder characterized by progressive loss of HCEnCs. We previously reported increased mitochondrial fragmentation and reduced ATP and mtDNA copy number in FECD. Herein, carbonyl cyanide m-chlorophenyl hydrazone (CCCP)-induced mitochondrial depolarization decreased mitochondrial mass and Mfn2 levels, which were rescued with mitophagy blocker, bafilomycin, in FECD...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28749896/elimination-of-anterior-corneal-steepening-with-descemet-membrane-endothelial-keratoplasty-in-a-patient-with-fuchs-dystrophy-and-keratoconus-implications-for-iol-calculation
#8
Reena Gupta, Ruta Kinderyte, Deborah S Jacobs, Ula V Jurkunas
PURPOSE: To report a case of coexistent Fuchs endothelial corneal dystrophy (FECD) and keratoconus (KCN) in which there was normalization of corneal topography after Descemet membrane endothelial keratoplasty (DMEK). METHODS: Retrospective medical record review. RESULTS: Preoperative findings revealed a best-corrected visual acuity of 20/40 with -1.00 - 2.50 × 147, topographic maximum keratometry of 50.8 D with inferior steeping, and confluent guttae in the left eye...
October 2017: Cornea
https://www.readbyqxmd.com/read/28742617/simple-single-pass-technique-for-ultrathin-descemet-stripping-automated-endothelial-keratoplasty-a-pilot-study
#9
Maria E S Dimitry, Adam D Lewis, Fani Zacharaki, Manju Chandran, Deana Robson, Geeta Menon, Thomas R G Poole
PURPOSE: To assess intraoperative and postoperative graft thickness (GT) after donor deturgescence for ultrathin Descemet stripping automated endothelial keratoplasty and to evaluate visual outcomes, endothelial cell density, and patient satisfaction at 1 year. METHODS: Prospective interventional case series of patients with Fuchs endothelial dystrophy, Fuchs endothelial dystrophy and cataract, and pseudophakic bullous keratopathy (n = 12 grafts). The donor cornea was allowed to thin out by simple evaporation on an artificial anterior chamber, to the required precut thickness, before a single microkeratome pass...
October 2017: Cornea
https://www.readbyqxmd.com/read/28738416/conditionally-immortal-slc4a11-mouse-corneal-endothelial-cell-line-recapitulates-disrupted-glutaminolysis-seen-in-slc4a11-mouse-model
#10
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
#11
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...
October 2017: Cornea
https://www.readbyqxmd.com/read/28727885/sustained-activation-of-the-unfolded-protein-response-induces-cell-death-in-fuchs-endothelial-corneal-dystrophy
#12
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
#13
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
#14
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 2017: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/28697678/ek-dlek-dsek-dmek-new-frontier-in-cornea-surgery
#15
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
#16
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
#17
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...
August 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
#18
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
#19
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
#20
COMPARATIVE STUDY
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
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