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Fuchs Endothelial Dystrophy

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Clinical Outcomes and Endothelial Cell Density After Descemet Membrane Endothelial Keratoplasty Using Peripherally-trephinated Donor Tissue (DMEK-pD) in Fuchs Endothelial Corneal Dystrophy.

Samar K Basak, Soham Basak,

PURPOSE:To evaluate the clinical outcomes and endothelial cell density (ECD) after Descemet membrane endothelial keratoplasty using peripherally trephinated donor tissue (DMEK-pD) and compare with DMEK using centrally trephinated donor tissue (DMEK-cD) in patients with Fuchs endothelial corneal dystrophy (FECD). METHODS:This was a prospective comparative interventional case series. One hundred twenty-five ... Read more >>

Cornea (Cornea)
[2020, 39(4):437-442]

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Start codon disruption with CRISPR/Cas9 prevents murine Fuchs’ endothelial corneal dystrophy

Hironori Uehara, Xiaohui Zhang, Felipe Pereira, Siddharth Narendran, Susie Choi, Sai Bhuvanagiri, Jinlu Liu, Sangeetha Ravi Kumar, Austin Bohner, Lara Carroll, Bonnie Archer, Yue Zhang, Wei Liu, Guangping Gao, Jayakrishna Ambati, Albert Jun, Balamurali Ambati,

Abstract A missense mutation of collagen type VIII alpha 2 chain (COL8A2) gene leads to early onset Fuchs’ endothelial corneal dystrophy (FECD), which progressively impairs vision through loss of corneal endothelial cells. We demonstrate that CRISPR/Cas9-based postnatal gene editing achieves structural and functional rescue in a mouse model of FECD. ... Read more >>

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Changing Trends in Penetrating Keratoplasty Indications between 2011 and 2018 - Histopathology of 2123 Corneal Buttons in a Single Center in Germany.

Milán Tamás Pluzsik, Berthold Seitz, Fidelis A Flockerzi, Achim Langenbucher, Gábor Tóth, Rainer M Bohle, Nóra Szentmáry,

Purpose: To analyse the changing trends in penetrating keratoplasty indications between January 2011 and December 2018, at the Department of Ophthalmology, Saarland University Medical Center, Homburg/Saar, Germany.Patients and Methods: This is a retrospective review of 2123 corneal buttons of 1776 patients (1993 eyes, 56.0% males, age at the time of ... Read more >>

Curr. Eye Res. (Current eye research)
[2020, :1-6]

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Long-term outcome of descemet membrane endothelial keratoplasty (DMEK) following failed penetrating keratoplasty (PK).

Silvia Schrittenlocher, Simona L Schlereth, Sebastian Siebelmann, Takahiko Hayashi, Mario Matthaei, Björn Bachmann, Claus Cursiefen,

PURPOSE:To assess the long-term outcome of Descemet membrane endothelial keratoplasty (DMEK) following failed penetrating keratoplasty (PK). METHODS:Retrospective review of 1840 consecutive DMEK surgeries from the prospective Cologne DMEK database performed between 07/2011 and 08/2017 at the Department of Ophthalmology, University of Cologne. RESULTS:Fifty-two eyes received a DMEK surgery after failed ... Read more >>

Acta Ophthalmol (Acta ophthalmologica)
[2020, :]

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Coexistence of Congenital Hereditary Endothelial Dystrophy and Fuchs Endothelial Corneal Dystrophy Associated With SLC4A11 Mutations in Affected Families.

Sunita Chaurasia, Muralidhar Ramappa, Mohini Annapurna, Chitra Kannabiran,

PURPOSE:To evaluate parents of probands affected with autosomal recessive congenital hereditary endothelial dystrophy (CHED) for clinical signs of Fuchs endothelial corneal dystrophy (FECD) and to determine the genotypes of the SLC4A11 gene in the probands and their parents. METHODS:This study involved 9 patients affected with CHED from 8 families. The ... Read more >>

Cornea (Cornea)
[2020, 39(3):354-357]

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Repeatability of Scheimpflug Tomography for Assessing Fuchs Endothelial Corneal Dystrophy.

Sanjay V Patel, David O Hodge, Emily J Treichel, Matthew R Spiegel, Keith H Baratz,

PURPOSE:To determine if agreement between subjective interpretations of Scheimpflug tomography maps of corneas with Fuchs endothelial corneal dystrophy (FECD) is affected by daily and hourly changes in corneal edema. DESIGN:Reliability analysis. METHODS:Scheimpflug imaging pachymetry and posterior elevation maps of corneas with a range of severity of FECD were evaluated in ... Read more >>

Am. J. Ophthalmol. (American journal of ophthalmology)
[2020, :]

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Quarter-Descemet Membrane Endothelial Keratoplasty: One- to Two-Year Clinical Outcomes.

Rénuka S Birbal, Sorcha Ni Dhubhghaill, Lamis Baydoun, Lisanne Ham, Vincent J A Bourgonje, Isabel Dapena, Silke Oellerich, Gerrit R J Melles,

PURPOSE:To report clinical outcomes of the first Quarter-Descemet membrane endothelial keratoplasty (Quarter-DMEK) case series performed for central Fuchs endothelial corneal dystrophy. METHODS:This is a prospective, interventional case series analyzing the clinical outcomes of 19 eyes of 19 patients with central Fuchs endothelial corneal dystrophy, that is, with guttae predominantly in ... Read more >>

Cornea (Cornea)
[2020, 39(3):277-282]

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Five-Year Graft Survival and Clinical Outcomes of 500 Consecutive Cases After Descemet Membrane Endothelial Keratoplasty.

Rénuka S Birbal, Sorcha Ni Dhubhghaill, Vincent J A Bourgonje, Jennifer Hanko, Lisanne Ham, Martine J Jager, Stefan Böhringer, Silke Oellerich, Gerrit R J Melles,

PURPOSE:To report the 5-year graft survival and clinical outcomes after Descemet membrane endothelial keratoplasty (DMEK). METHODS:A retrospective, interventional case series was performed at a tertiary referral center. Five hundred eyes of 393 patients that underwent DMEK for Fuchs endothelial corneal dystrophy, bullous keratopathy, failed previous corneal transplants other than DMEK, ... Read more >>

Cornea (Cornea)
[2020, 39(3):290-297]

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Delivery of Antisense Oligonucleotides to the Cornea.

Viet Q Chau, Jiaxin Hu, Xin Gong, John D Hulleman, Rafael L Ufret-Vincenty, Frank Rigo, Thahza P Prakash, David R Corey, V Vinod Mootha,

Antisense oligonucleotides (ASOs) are synthetic nucleic acids that recognize complementary RNA sequences inside cells and modulate gene expression. In this study, we explore the feasibility of ASO delivery to the cornea. We used quantitative polymerase chain reaction to test the efficacy of a benchmark ASO targeting a noncoding nuclear RNA, ... Read more >>

Nucleic Acid Ther (Nucleic acid therapeutics)
[2020, :]

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A Family of Fuchs Endothelial Corneal Dystrophy and Anterior Polar Cataract with An Analysis of Whole Exome Sequencing

Hong Zhang, Xue Jiang, Xin Jin, Nan Zhang,

<title>Abstract</title> <p> <bold>Background</bold> Our aim was to introduce a family affected by this rare phenotype, and perform the whole exome sequencing (WES) to explore the potential candidate genes causing the disorders.<bold>Methods </bold>A five-generation family including 5 patients affected by FECD with APC, and 9 patients suffered from only ... Read more >>

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[The German version of the Visual Function and Corneal Health Status (V‑FUCHS): a Fuchs dystrophy-specific visual disability instrument].

Viviane Grewing, Marianne Fritz, Claudia Müller, Daniel Böhringer, Thomas Reinhard, Sanjay V Patel, Katrin Wacker,

BACKGROUND:In Fuchs' endothelial corneal dystrophy (FECD), the timing and expectation of interventions has changed in recent years because of improved therapeutic options. To assess outcomes and compare interventions, a patient-reported quantification of visual disability is important. OBJECTIVE:To develop the German version of the Visual Function and Corneal Health Status (V‑FUCHS), an ... Read more >>

Ophthalmologe (Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft)
[2020, 117(2):140-146]

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Microscope Integrated Intraoperative Optical Coherence Tomography-Guided DMEK in Corneas with Poor Visualization.

Namrata Sharma, Pranita Sahay, Prafulla K Maharana, Praveen Kumar, Saima Ahsan, Jeewan S Titiyal,

Purpose:To assess the utility of microscope-integrated intraoperative optical coherence tomography (Mi-OCT) for performing Descemet membrane endothelial keratoplasty (DMEK) in corneas with poor visualization. Methods:It is a prospective interventional case series that included 25 consecutive cases of corneal decompensation with poor visualization that underwent Mi-OCT-guided DMEK at a tertiary eye care ... Read more >>

Clin Ophthalmol (Clinical Ophthalmology (Auckland, N.Z.))
[2020, 14:643-651]

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Unilateral acute iris depigmentation and transillumination after glaucoma surgery with mitomycin application and intracameral moxifloxacin.

Carmen Sánchez-Sánchez, Beatriz Puerto, Cristina López-Caballero, Inés Contreras,

Purpose:Bilateral acute iris depigmentation (BADI) and transillumination (BATI) syndromes have been linked with the use of antibiotics, especially fluoroquinolones. They are characterized by acute onset of pigment dispersion in the anterior chamber, depigmentation of the iris and pigment deposition in the angle and in the posterior surface of the cornea ... Read more >>

Am J Ophthalmol Case Rep (American journal of ophthalmology case reports)
[2020, 18:100639]

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Author Correction: Aberrant DNA methylation of miRNAs in Fuchs endothelial corneal dystrophy.

Peipei Pan, Daniel J Weisenberger, Siyu Zheng, Marie Wolf, David G Hwang, Jennifer R Rose-Nussbaumer, Ula V Jurkunas, Matilda F Chan,

An amendment to this paper has been published and can be accessed via a link at the top of the paper. ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):2395]

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5-Year Outcomes of Descemet Stripping Only in Fuchs Dystrophy.

June Artaechevarria Artieda, Matthew Wells, Raj N Devasahayam, Gregory Moloney,

PURPOSE:To report the 5-year outcomes of the first reported case of bilateral Descemet stripping only (DSO) for Fuchs endothelial corneal dystrophy (FECD) at our center. Visual, biomicroscopic, and confocal microscopic findings are described. METHODS:A retrospective case report. RESULTS:A 55-year-old woman with bilateral FECD was referred with decreased visual acuity and ... Read more >>

Cornea (Cornea)
[2020, :]

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Ex vivo excimer laser ablation of cornea guttata and ROCK inhibitor-aided endothelial recolonization of ablated central cornea.

Stefan Kassumeh, Annabel von Studnitz, Siegfried G Priglinger, Rudolf Fuchshofer, Nikolaus Luft, Gregory Moloney, Martin Dirisamer, Andreas Ohlmann,

PURPOSE:To determine whether excimer laser ablation of guttae is a viable strategy for removal of diseased tissue in Fuchs' endothelial corneal dystrophy (FECD) on excised human Descemet membranes and whether an excimer laser-created wound on healthy human corneas ex vivo is recolonized with corneal endothelial cells. METHODS:Descemet membranes of FECD ... Read more >>

Acta Ophthalmol (Acta ophthalmologica)
[2020, :]

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Refractive lens exchange with a trifocal intraocular lens in Fuchs endothelial dystrophy.

Mayank A Nanavaty, Zahra Ashena,

A 57-year-old man with myopia presented with advanced Fuchs endothelial dystrophy, a refraction of -6.00 diopters (D) and -6.25 D, an endothelial cell count of 1100 cells/mm and 1000 cells/mm in right and left eyes, respectively, and corrected distance visual acuity of 20/30 in each eye. The patient was deemed ... Read more >>

J Cataract Refract Surg (Journal of cataract and refractive surgery)
[2020, 46(3):478-481]

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Brillouin Microscopy Visualizes Centralized Corneal Edema in Fuchs Endothelial Dystrophy.

Amira M Eltony, Fatima Clouser, Peng Shao, Roberto Pineda, Seok-Hyun Yun,

PURPOSE:To investigate the feasibility of using Brillouin microscopy for assessment of corneal edema in patients with Fuchs endothelial corneal dystrophy (FECD). Brillouin microscopy analyzes the frequency shift of light inelastically scattered by naturally occurring acoustic waves in a small volume of tissue. The resulting frequency shift is a measure of ... Read more >>

Cornea (Cornea)
[2020, 39(2):168-171]

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Five-year follow-up outcomes after Descemet's stripping automated endothelial keratoplasty: a retrospective study.

Koichi Wakimasu, Koji Kitazawa, Kanae Kayukawa, Isao Yokota, Tsutomu Inatomi, Osamu Hieda, Chie Sotozono, Shigeru Kinoshita,

Objective:To assess graft survival and endothelial cell density (ECD) over a 5-year follow-up period after Descemet's stripping automated endothelial keratoplasty (DSAEK). Methods and analysis:This retrospective study involved 130 eyes of 130 consecutive patients with Fuchs endothelial corneal dystrophy (FECD), non-FECD bullous keratopathy (BK) (non-FECD) or BK post-trabeculectomy or tube-shunt surgery ... Read more >>

BMJ Open Ophthalmol (BMJ open ophthalmology)
[2020, 5(1):e000354]

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Five Novel Mutations in LOXHD1 Gene Were Identified to Cause Autosomal Recessive Nonsyndromic Hearing Loss in Four Chinese Families.

Xiaohui Bai, Chi Zhang, Fengguo Zhang, Yun Xiao, Yu Jin, Haibo Wang, Lei Xu,

Hearing loss is one of the most common sensory disorders in newborns and is mostly caused by genetic factors. Autosomal recessive nonsyndromic hearing loss (ARNSHL) is usually characterized as a severe-to-profound congenital sensorineural hearing loss and later can cause various degrees of defect in the language and intelligent development of ... Read more >>

Biomed Res Int (BioMed Research International)
[2020, 2020:1685974]

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In Vitro Evaluation of the Feasibility of Slit-Lamp Nd: YAG Laser Descemetorhexis.

Diana C Dragnea, Sorcha Ní Dhubhghaill, Vincent J A Bourgonje, Isabel Dapena, Silke Oellerich, Gerrit R J Melles, Itay Lavy,

PURPOSE:To evaluate in vitro the feasibility and tissue effects of using a slit-lamp neodymium-doped yttrium aluminum garnet (Nd:YAG) laser to create a central descemetorhexis in human donor corneas. METHODS:Twelve human donor corneas ineligible for transplantation were divided into 2 groups, A and B. Group A: 2 "healthy" corneas, which were ... Read more >>

Cornea (Cornea)
[2020, 39(2):229-233]

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Analyzing Presymptomatic Tissue to Gain Insights into the Molecular and Mechanistic Origins of Late-Onset Degenerative Trinucleotide Repeat Disease

Yongjun Chu, Jiaxin Hu, Hanquan Liang, Mohammed Kanchwala, Chao Xing, Walter Beebe, Charles Bowman, Xin Gong, David Corey, Vinod Mootha,

ABSTRACT How genetic defects trigger the molecular changes that cause late-onset disease is important for understanding disease progression and therapeutic development. Fuchs’ endothelial corneal dystrophy (FECD) is an RNA-mediated disease caused by a trinucleotide CUG expansion in an intron within the TCF4 gene. The mutant intronic CUG RNA is present ... Read more >>

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Morning myopic shift and glare in advanced Fuchs endothelial corneal dystrophy.

Niklas Loreck, Werner Adler, Sebastian Siebelmann, Alexander C Rokohl, Ludwig M Heindl, Claus Cursiefen, Björn O Bachmann, Mario Matthaei,

PURPOSE:Impaired subjective morning visual acuity with improvement of symptoms during the day is pathognomonic for corneal endothelial dysfunction in advanced Fuchs endothelial corneal dystrophy (FECD). This study aimed to analyze the daily fluctuations of corneal thickness, refraction, and (glare) visual acuity in advanced FECD. DESIGN:Prospective cohort study. METHODS:Patients with advanced ... Read more >>

Am. J. Ophthalmol. (American journal of ophthalmology)
[2020, :]

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Placoid choroidopathy after bilateral uncomplicated descemet's membrane endothelial keratoplasty.

Thomas H Dohlman, Neepa Shah, Colin Prensky, Mrinali P Gupta, Donald J D'Amico, Szilard Kiss, Christopher S Sales,

Purpose:To describe a case of bilateral, sequential placoid choroidopathy following uncomplicated Descemet's membrane endothelial keratoplasty (DMEK). Observations:A 49-year old woman presented with flashing lights and central visual field scotomas after undergoing uncomplicated DMEK combined with cataract surgery for Fuch's endothelial dystrophy in the right eye. She was found to have ... Read more >>

Am J Ophthalmol Case Rep (American journal of ophthalmology case reports)
[2020, 17:100610]

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Diagnostic Performance of 3-Dimensional Thickness of the Endothelium-Descemet Complex in Fuchs' Endothelial Cell Corneal Dystrophy.

Taher Eleiwa, Amr Elsawy, Mohamed Tolba, William Feuer, Sonia Yoo, Mohamed Abou Shousha,

PURPOSE:To describe the diagnostic accuracy of 3-dimensional (3D) endothelium-Descemet's membrane complex thickness (En-DMT) in Fuchs' endothelial corneal dystrophy (FECD) and determine its potential role as an objective index of disease severity. DESIGN:Observational case-control study. PARTICIPANTS:One hundred four eyes of 79 participants (64 eyes of 41 FECD patients and 40 eyes ... Read more >>

Ophthalmology (Ophthalmology)
[2020, :]

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