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Fuchs' corneal dystrophy

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https://www.readbyqxmd.com/read/29136408/fuchs-endothelial-and-myotonic-dystrophies-corneal-dystrophy-in-myotonic-patients
#1
Maddalena De Bernardo, Nicola Rosa
No abstract text is available yet for this article.
November 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29132691/-practical-assessment-of-dsaek-in-the-management-of-endothelial-decompensation-following-penetrating-keratoplasty
#2
E Neiter, C Goetz, F Tortuyaux, A Ehrhardt, N Houmad, J-M Perone
PURPOSE: To report our anatomical and functional results as well as possible complications of the first six Descemet's stripping endothelial keratoplasties (DSAEK) performed in our department for endothelial decompensation after penetrating keratoplasty (PK) METHODS: This was a retrospective and observational monocentric study of six patients with DSAEK after prior PK between January 2015 and July 2016. The data collected were: demographic characteristics (age, sex), ophthalmological comorbidities, initial indication for PK, delay between PK and DSAEK...
November 11, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29127432/corneal-endothelial-cell-loss-3-years-after-successful-descemet-stripping-automated-endothelial-keratoplasty-in-the-cornea-preservation-time-study-a-randomized-clinical-trial
#3
Jonathan H Lass, Beth Ann Benetz, David D Verdier, Loretta B Szczotka-Flynn, Allison R Ayala, Wendi Liang, Anthony J Aldave, Steven P Dunn, Tyrone McCall, Shahzad I Mian, Lisa C Navarro, Sanjay V Patel, Sudeep Pramanik, George O Rosenwasser, Kevin W Ross, Mark A Terry, Craig Kollman, Robin L Gal, Roy W Beck
Importance: Demonstrating that endothelial cell loss following Descemet stripping automated endothelial keratoplasty (DSAEK) is independent of donor cornea preservation time (PT) could increase the pool of corneal tissue available for keratoplasty. Objective: To determine whether endothelial cell loss 3 years after successful DSAEK is related to PT. Design, Setting, and Participants: A multicenter, double-masked, randomized clinical trial included 40 clinical sites (70 surgeons) in the United States, with donor corneas provided by 23 US eye banks...
November 10, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/29127431/effect-of-cornea-preservation-time-on-success-of-descemet-stripping-automated-endothelial-keratoplasty-a-randomized-clinical-trial
#4
George O Rosenwasser, Loretta B Szczotka-Flynn, Allison R Ayala, Wendi Liang, Anthony J Aldave, Steven P Dunn, Tyrone McCall, Lisa C Navarro, Sudeep Pramanik, Kevin W Ross, R Doyle Stulting, Mark A Terry, Elmer Y Tu, David D Verdier, Craig Kollman, Robin L Gal, Roy W Beck, Jonathan H Lass
Importance: Demonstrating that success of Descemet stripping automated endothelial keratoplasty is similar across donor cornea preservation times (PTs) could increase the donor pool. Objective: To determine whether the 3-year rate of graft success using corneal donor tissue preserved 8 to 14 days is noninferior to that of donor tissue preserved 7 days or less. Design, Setting, and Participants: A multicenter, double-masked, randomized noninferiority clinical trial was conducted from April 16, 2012, to June 5, 2017, at 40 clinical sites (70 surgeons) in the United States, with donor corneas provided by 23 US eye banks...
November 10, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/29120722/recurrent-endothelial-rejection-after%C3%A2-dsaek-and-dmek-causing-corneal-perforation-in-a-patient-with-history-of%C3%A2-fuchs%C3%A2-endothelial-dystrophy-october-consultation-1
#5
Majid Moshirfar
No abstract text is available yet for this article.
October 2017: Journal of Cataract and Refractive Surgery
https://www.readbyqxmd.com/read/29077583/phenotypic-characterization-of-corneal-endothelial-dystrophy-in-german-shorthaired-and-wirehaired-pointers-using-in-vivo-advanced-corneal-imaging-and-histopathology
#6
Olivia R Shull, Christopher M Reilly, Lola B Davis, Christopher J Murphy, Sara M Thomasy
PURPOSE: To evaluate corneal morphology using ultrasonic pachymetry (USP), Fourier-domain optical coherence tomography (FD-OCT), and in vivo confocal microscopy (IVCM) in 2 related canine breeds-German shorthaired pointers (GSHPs) and German wirehaired pointers (GWHPs)-with and without corneal endothelial dystrophy (CED). This condition is characterized by premature endothelial cell degeneration leading to concomitant corneal edema and is similar to Fuchs endothelial corneal dystrophy...
October 25, 2017: Cornea
https://www.readbyqxmd.com/read/29056323/impeding-transcription-of-expanded-microsatellite-repeats-by-deactivated-cas9
#7
Belinda S Pinto, Tanvi Saxena, Ruan Oliveira, Héctor R Méndez-Gómez, John D Cleary, Lance T Denes, Ona McConnell, Juan Arboleda, Guangbin Xia, Maurice S Swanson, Eric T Wang
Transcription of expanded microsatellite repeats is associated with multiple human diseases, including myotonic dystrophy, Fuchs endothelial corneal dystrophy, and C9orf72-ALS/FTD. Reducing production of RNA and proteins arising from these expanded loci holds therapeutic benefit. Here, we tested the hypothesis that deactivated Cas9 enzyme impedes transcription across expanded microsatellites. We observed a repeat length-, PAM-, and strand-dependent reduction of repeat-containing RNAs upon targeting dCas9 directly to repeat sequences; targeting the non-template strand was more effective...
November 2, 2017: Molecular Cell
https://www.readbyqxmd.com/read/29053562/predictive-factors-for-corneal-clearance-after-descemetorhexis-without-endothelial-keratoplasty
#8
Emma Davies, Ula Jurkunas, Roberto Pineda
PURPOSE: To study preoperative factors that possibly contribute to corneal clearance after Descemetorhexis without endothelial keratoplasty (DWEK) and to determine the most successful surgical technique for the procedure. METHODS: Retrospective chart review of 17 eyes of 13 patients with Fuchs corneal endothelial dystrophy that underwent central 4-mm DWEK. RESULTS: Baseline age, pachymetry, and endothelial cell count were not statistically significantly different between eyes that cleared 0 to 2 months, 3 to 5 months, 6 to 8 months, or that failed to clear...
October 17, 2017: Cornea
https://www.readbyqxmd.com/read/29044056/association-of-polymorphisms-in-the-intron-of-tcf4-gene-to-late-onset-fuchs-endothelial-corneal-dystrophy-an-indian-cohort-study
#9
Bhavna S Rao, Arokiasamy Tharigopala, Sudhir R Rachapalli, Rama Rajagopal, Nagasamy Soumittra
PURPOSE: Fuchs endothelial corneal dystrophy (FECD) is a progressive degenerative disease of the corneal endothelium. It is genetically heterogeneous and follows either an autosomal dominant or sporadic pattern of inheritance. Here, we have explored the association of four previously reported intronic single nucleotide polymorphisms and intronic CTG repeat expansions in TCF4 gene to FECD in an Indian cohort. METHODS: The cohort consisting of 52 sporadic late-onset cases, 5 early-onset cases, and 148 controls was taken for the study...
October 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29040117/factors-limiting-the-visual-outcome-after-descemet-stripping-automated-endothelial-keratoplasty-comprehensive-analysis-including-the-graft-position-and-irregularity
#10
Yasuaki Iwama, Takeshi Soma, Naoyuki Maeda, Yuzuru Sasamoto, Yoshinori Oie, Shizuka Koh, Kohji Nishida
PURPOSE: To investigate factors affecting best postoperative corrected distance visual acuity (CDVA) in patients who have undergone Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: Forty-two eyes of 42 patients with predominantly non-Fuchs endothelial corneal dystrophy after DSAEK were retrospectively evaluated. All patients were examined using anterior segment optical coherence tomography when their best postoperative CDVA had been achieved...
October 13, 2017: Cornea
https://www.readbyqxmd.com/read/29040116/air-versus-sulfur-hexafluoride-gas-tamponade-in-descemet-membrane-endothelial-keratoplasty-a-fellow-eye-comparison
#11
Philipp V von Marchtaler, Julia M Weller, Friedrich E Kruse, Theofilos Tourtas
PURPOSE: To perform a fellow eye comparison of outcomes and complications when using air or sulfur hexafluoride (SF6) gas as a tamponade in Descemet membrane endothelial keratoplasty (DMEK). METHODS: One hundred thirty-six eyes of 68 consecutive patients who underwent uneventful DMEK in both eyes for Fuchs endothelial corneal dystrophy were included in this retrospective study. Inclusion criteria were air tamponade (80% of the anterior chamber volume) in the first eye and 20% SF6 gas tamponade (80% of the anterior chamber volume) in the second eye; and same donor tissue culture condition in both eyes...
October 13, 2017: Cornea
https://www.readbyqxmd.com/read/28976417/parents-of-patients-with-congenital-hereditary-endothelial-dystrophy-should-be-evaluated-for-fuchs-endothelial-corneal-dystrophy
#12
Sunita Chaurasia, Muralidhar Ramappa, Chitra Kannabiran
No abstract text is available yet for this article.
December 2017: Cornea
https://www.readbyqxmd.com/read/28968295/descemet-membrane-endothelial-transfer-ultimate-outcome
#13
Rénuka S Birbal, Jack Parker, Martin Dirisamer, Ana Janićijević, Lamis Baydoun, Isabel Dapena, Gerrit R J Melles
PURPOSE: To evaluate the clinical outcome of 16 eyes undergoing Descemet membrane endothelial transfer (DMET). METHODS: In this retrospective cohort study, a consecutive series of 16 eyes from 16 patients was evaluated after subtotal detachment of the Descemet graft after a Descemet membrane endothelial keratoplasty procedure (n = 8) or intended DMET (n = 8) for either Fuchs endothelial dystrophy (n = 10) or bullous keratopathy (BK; n = 6). RESULTS: All 8 Descemet membrane endothelial keratoplasty procedures were complicated by subtotal detachment of the donor graft...
September 29, 2017: Cornea
https://www.readbyqxmd.com/read/28886202/fuchs-endothelial-corneal-dystrophy-and-rna-foci-in-patients-with-myotonic-dystrophy
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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