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Endothelial corneal dystrophy

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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
#1
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...
July 26, 2017: Cornea
https://www.readbyqxmd.com/read/28742617/simple-single-pass-technique-for-ultrathin-descemet-stripping-automated-endothelial-keratoplasty-a-pilot-study
#2
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...
July 24, 2017: Cornea
https://www.readbyqxmd.com/read/28738416/conditionally-immortal-slc4a11-mouse-corneal-endothelial-cell-line-recapitulates-disrupted-glutaminolysis-seen-in-slc4a11-mouse-model
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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/28683480/-penetrating-limbokeratoplasty-for-gelatinous-corneal-dystrophy
#9
Stefan J Lang, Daniel Böhringer, Thomas Reinhard
Background Gelatinous corneal dystrophy is a very rare disease in Europe and North America. Patients suffer from pain, photophobia and loss of vision, usually before the age of 20. Transplantation of limbal stem cells was proposed due to the high rate of recurrence after conventional penetrating keratoplasty. We present the results of penetrating limbokeratoplasty, a combined transplantation of cornea and limbal stem cells, in patients with gelatinous corneal dystrophy. Methods We present a series of 7 consecutive eyes with gelatinous corneal dystrophy, which were treated with penetrating limbokeratoplasty-4 eyes of 3 patients had already undergone multiple conventional corneal transplantations...
July 6, 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28654985/transcriptomic-profiling-of-posterior-polymorphous-corneal-dystrophy
#10
Doug D Chung, Ricardo F Frausto, Benjamin R Lin, Evelyn M Hanser, Zack Cohen, Anthony J Aldave
Purpose: To investigate the molecular basis of posterior polymorphous corneal dystrophy (PPCD) by examining the PPCD transcriptome and the effect of decreased ZEB1 expression on corneal endothelial cell (CEnC) gene expression. Methods: Next-generation RNA sequencing (RNA-seq) analyses of corneal endothelium from two PPCD-affected individuals (one with PPCD3 and one of unknown genetic cause) compared with two age-matched controls, and primary human CEnC (pHCEnC) transfected with siRNA-mediated ZEB1 knockdown...
June 1, 2017: Investigative Ophthalmology & Visual 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
#11
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/28624226/transcriptome-wide-investigation-of-mrna-circrna-in-mir-184-and-its-r-57c-u-mutant-type-treatment-of-human-lens-epithelial-cells
#12
Yueqiu Luo, Siyu Liu, Ke Yao
m-miR-184 (mutant miR-184, r.57c > u) appears in familial hereditary ocular diseases, including keratoconus, cataracts, EDICT (endothelial dystrophy, iris hypoplasia, congenital cataract, and stromal thinning) syndrome, severe keratoconus, and non-ectatic corneal thinning. The biological function of m-miR-184 in these ocular diseases remains unclear. With the emergence of high-throughput sequencing, it is now possible to discover many different biological components simultaneously. Using two different RNA libraries, we sequenced the complete transcriptome of HLE cells treated with miR-184, m-miR-184, and a negative control...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28623041/-corneal-dystrophies
#13
REVIEW
J-L Bourges
Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09 % of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. Practically, the ophthalmologist manages functional symptoms, such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses...
June 13, 2017: Journal Français D'ophtalmologie
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
#14
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/28606270/-keratoplasty-classification-and-primary-disease-spectrum-analysis-of-315-cases
#15
S B Cai, M Sun, S Li, L J Xu, W Wang, J Wang, W K Hu, X Y Li, P Wang, H Zhang, G G Li
Objective: To identify the primary disease spectrum and trends of surgical procedure of keratoplasty patients. Methods: Retrospective case series study. To review all patients who underwent keratoplasty at Department of ophthalmology in Tongji Hospital from January 1, 2012 to December 31, 2015. The data collected included age, sex, birthplace, and primary corneal disease and associated surgical procedures. Then the data were compared with similar papers domestic and foreign. Results: A total of 315 keratoplasties were performed during this 4-year period...
June 11, 2017: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/28600303/stability-of-visual-outcome-between-2-and-5-years-following-corneal-transplantation-in-the-uk
#16
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
#17
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
#18
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/28583694/corneal-dystrophies
#19
J-L Bourges
Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09% of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. In practice, the ophthalmologist manages functional symptoms such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses...
June 2, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28559836/unilateral-posterior-polymorphous-corneal-dystrophy-presented-as-anisometropic-astigmatism-3-case-reports
#20
Hyun Sun Jeon, Joon Young Hyon
BACKGROUND: Posterior polymorphous corneal dystrophy (PPCD) is typically considered bilateral and asymptomatic. However, few case reports on patients with unilateral PPCD with asymmetric refractive error have mentioned anisometropic amblyopia development. In support of this, we report 3 cases of unilateral PPCD that presented as anisometropic astigmatism. Visual prognosis related to amblyopia development is discussed. CASE PRESENTATION: All 3 patients had a band lesion in the affected eye and a difference of at least 1...
January 2017: Case Reports in Ophthalmology
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