bluuebunny/crossref_metadata_2025_split huggingface dataset:
Human induced pluripotent stem cell‐derived retinal pigment epithelial cell model of Sorsby fundus dystrophy
Authors: Hongisto Heidi 
Published: December 2019

AbstractSorsby fundus dystrophy (SFD) is a rare degenerative disease of the macular retina, caused by mutations in the tissue inhibitor of matrix metalloproteinase‐3 (TIMP3) gene. SFD leads to bilateral loss of central vision and shows significant clinical overlap with the more common age‐related macular degeneration, suggesting shared underlying mechanisms. In SFD the mutant TIMP‐3 deposits under the retinal pigment epithelium (RPE), leading to pathological sub‐RPE thickening, but the initiating pathology is poorly understood. We have established patient specific human induced pluripotent stem cell (hiPSC) lines from three SFD patients carrying Ser204Cys mutation in TIMP3 gene, and three healthy controls. The hiPSC lines were extensively characterized and differentiated to RPE. SFD‐iPSC‐RPE showed no difference in RPE properties such as morphology, gene or protein expression, phagocytosis capacity or growth factor secretion compared to control‐hiPSC‐RPE. SFD‐iPSC‐RPE showed lowered epithelial resistance, and transmission electron microscopy indicated basal laminar changes and deposits under the SFD‐iPSC‐RPE. Quantitative proteomics analyses indicated upregulation of retinal degeneration associated pathways and accumulation of related proteins including apolipoprotein E. Western blotting verified clear accumulation of the TIMP3 protein in the SFD‐iPSC‐RPE cultures. Overall, the SFD‐iPSC‐RPE cell models recapitulate the disease phenotype in vitro for the study of disease mechanisms.
History of concepts of myopia pathogenesis and treatment
Authors: Grzybowski Andrzej 
Published: December 2019

AbstractIn the last 60 years, there has been a marked increase in the prevalence of high myopia in developed countries in East and Southeast Asia, and there are signs of similar, but less dramatic increases, in North America and Europe. In some parts of the world, 70‐90% of children completing high schools are myopic, and as many as 20% may be highly myopic. It is accepted now that myopia results from excessive axial elongation of the eye, which appears to be environmentally driven. The present methods of myopia control are based on the use of topical atropine and increased outdoor activity. Interestingly, the early observations on similar concepts and the first reports of atropine treatment date back to the 19th century. This is evidenced by papers of Ware (1813), Cohn (1864), and Tscherning (1883). Dobrowolsky published the first case series case series of myopia improvement after atropine therapy in German in 1868.
High resolution imaging of radiation retinopathy
Authors: Paques Michel 
Published: December 2019

AbstractWe explored by fundus photographs, OCT and adaptive optics ophthalmoscopy (AOO) the vessels of eyes after proton beam therapy for uveal melanoma in 10 eyes. We observed strong fluctuations of diameters in both arteries and veins, without significant changes in vascular wall thickness suggesting that vasoconstriction was involved in this process. Reduction of lumen diameter could be focal or diffuse, and could lead in some cases to branch retinal vein occlusion unrelated to arteriovenous crossings. The ensuing collateral circulation could lead to the development of TelCaps (capillary macroaneurysms), successfully treated by targeted laser in one case. Many areas of capillary closure followed a vascular perfusion or drainage scheme, suggesting that vasoconstriction played a role in the complex pattern of radiation retinopathy. This may provide the basis for therapeutic trials based on the modulation of vasomotricity.
Autoinflammatory uveitis
Authors: Chaques‐Alepuz Vicente 
Published: December 2019

AbstractAutoinflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto‐antibodies or auto‐reactive T cells, and an inborn error of innate immunity.Ocular involvement is frequent in the monogenic autoinflammatory disorders and generally occurs as spontaneously recurring inflammatory events at different ocular sites caused by aberrant release of proinflammatory cytokines, mainly IL‐1β.Innate and adaptive immune systems have evolved together to protect the host by quickly responding to danger signals, eliminating pathogens and creating immunological memory as well as immunological tolerance to self. Loss of control mechanisms and/or regulation of either the adaptive or the innate immune system lead to autoimmunity and autoinflammation respectively.We present the clinical case of a family with three members affected with the same form of uveitis, that we have considered as presumed autoinflammatory uveitis.As from this case we discuss about pathogenesis and therapy of autoinflammatory diseases and uveitis.
Tear proteome in uveitis
Authors: Roger Eidet Jon 
Published: December 2019

AbstractPurposeUveitis represents the fourth commonest cause of legal blindness in adult patients. Analysis of tear fluid proteins in acute anterior uveitis (AAU) patients could represent a novel diagnostic modality. This study aimed at detecting potential tear fluid biomarkers in patients with AAU and bacterial keratitis (BK).MethodsFourteen patients with unilateral AAU, seven patients with bacterial keratitis (BK) and 14 healthy age‐ and sex‐matched control subjects were included. Tear fluid samples were obtained by inserting two Schirmer’s test strips behind the lower eyelid of each eye for five minutes without anesthesia. Proteins were then identified by liquid chromatography tandem mass spectrometry. Pooled tear fluid samples from healthy controls were compared with samples from AAU and BK patients, respectively. Ingenuity Pathway Analysis (IPA) was used to explore biological function.ResultsA relative protein level was obtained for 235 proteins in AAU patients and 198 proteins in BK patients, while using healthy control subjects as reference. In AAU patients, nine proteins were at least two‐fold up‐regulated, whereas in BK patients, 12 proteins were up‐regulated more than two‐fold. None of the top ten upregulated proteins in AAU and BK overlapped. The top canonical pathway identified by IPA was ‘Acute Phase Response Signaling’ in AAU and ‘LXR/RXR activation’ in BK. Top upstream activated regulators included peroxisome proliferator activated receptor delta in AAU and interleukin 6 in BK.ConclusionsTear fluid protein analysis represents a promising diagnostic modality for ocular inflammatory diseases and demonstrate differentially expressed proteins in acute anterior uveitis and bacterial keratitis.
Harnessing the power of OCT imaging in clinic by smart data analysis and presentation
Authors: Yap Timothy 
Published: December 2019

AbstractThe limiting factor in the success of imaging is the end‐user’s interpretation of it. Therefore, the manner in which the raw data is analysed and presented is crucial in leading the clinician to make the correct management decisions for each individual patient. With such a variety of OCT biomarkers and graphical representations available from a single scan, the results can often be time consuming for the clinician to analyse, underused due to lack of training or education, and often conflicting in terms of results. We aim to offer a summary of what we have available to us today in terms of the different ways OCT data is analysed and presented, and the clinical information that can be gleaned from these. Following this, we will explore novel ways in which OCT data can be most effectively presented in clinic and how this may help us make better, more efficient clinical decisions in order to improve the care for patients and meet the growing demands for glaucoma care.
Strategies to improve corneal endothelial graft outcomes
Authors: Levis Hannah 
Published: December 2019

AbstractOur research is focused on the development of strategies to improve corneal endothelial graft survival. We are interested in improving the current treatments by optimising endothelial graft preparation techniques, shipping and delivery protocols. We are also interested in combining cultured endothelial cells with novel biomaterials to create tissue engineered biosynthetic grafts suitable for transplantation and have developed a useful ex vivo corneal model to test these grafts before future in vivo experiments.
Abnormalities of vision and colour vision in Parkinson's disease
Authors: Weil Rimona 
Published: December 2019

AbstractOften thought of as a movement disorder, Parkinson's disease has prominent non‐motor symptoms. Vision is frequently affected, with deficits in acuity, contrast sensitivity and higher order visual processes, as well as retinal changes. These visual deficits may be predictive of dementia in Parkinson's disease. There is now pressure to define accurate markers that predict dementia, to help stratify clinical trials of disease modifying therapies in Parkinson's’ disease.We have collected visual, cognitive and retinal (OCT) data in 112 patients with Parkinson's and 35 age‐matched controls. We assessed risk of dementia using validated risk algorithms. We show that higher risk of dementia is associated with poorer performance in visual tests including in colour vision. We also show that retinal thinning in the ganglion cell and inner plexiform layers is linked with higher risk of dementia.Our findings suggest that visual measures and retinal structure may be useful to predict dementia in Parkinson's disease.
Limbal stem cell transplantation and keratoprosthesis
Authors: Moussa Sarah 
Published: December 2019

AbstractLimbal stem deficiency (LSCD) is a severe ocular disease which may lead to vision impairment or corneal blindness. Beside conservative options like topical and systemical medical therapy, chronic stage treatment options include conjunctival replacement procedures and limbal autografting. If limbal cell transplanation in form of either cultivated limbal epithelial transplantation (CLET), simple limbal epithelial transplantation (SLET) or conjunctival limbal autograft (CLAU) and corneal transplantation are not successful a Keratoprosthesis in form of a Boston Keratoprosthesis or a Osteo‐odonto‐ Keratoprosthesis might be the last chance for these patients. In the following presentation indication and surgical options in LSCD will be discussed.
OCT in peripheral chorioretinal tumors and pseudo‐tumors
Authors: Zografos Leonidas 
Published: December 2019

AbstractDifferential diagnosis of peripheral tumors and pseudo‐tumors may be challenging in selected cases. Peripheral OCT allows the location of the tumors in various chorioretinal layers and the recognition of internal reflectivity of the tumors. In addition, the presence and extent of the serous retinal detachment is documented in order to observe the evolution in the time. Peripheral OCT contributes in addition to the definition of tumor borders and assists the treatment planning for radiation therapy. The use of peripheral OCT is a valuable diagnostic modality for the diagnosis and management of peripheral tumors and pseudo‐tumors.
Bioengineered corneal stroma: from laboratory to clinical adoption
Authors: Rafat Mehrdad 
Published: December 2019

AbstractBlindness caused by corneal stromal disease and injuries affects millions worldwide, with the greatest burden in low‐ to middle‐income countries with inadequate donation and tissue banking infrastructure to meet the demand for sight‐restoring corneal transplantation. We bioengineered a regenerative cell‐free corneal scaffold from medical‐grade type I collagen and developed a minimally‐invasive implantation method avoiding the need for surgical suturing or long‐term immunosuppression. Applying good manufacturing practices and third‐party certified biocompatibility, toxicity, sterility and long‐term shelf life studies, sterile corneal scaffolds were tested in preclinical animal models and in a first clinical case series. Scaffolds integrated with recipient tissue and supported host cell ingrowth without inflammation or scarring. 22 subjects with advanced keratoconus were implanted with the bioengineered scaffold without complications. During 6–12 months of follow‐up, no rejection, inflammation, vascularization or adverse event occurred. Visual acuity, spherical refractive error, keratometry, corneal thickness and volume all improved significantly in the cohorts. Initially contact lens‐intolerant patients were tolerant postoperatively and are eligible for refractive surgery to optimize visual acuity. Results indicate bioengineered scaffold implantation can restore vision in patients with advanced stromal disease, avoiding more invasive procedures and the need for donor tissue.
Analysis of current colour assessment protocols for the cabin crew
Authors: Ozpinar Ayse 
Published: December 2019

AbstractColour vision (CV) requirements for cabin crewmembers are set in accordance with the regulations of the European Aviation Safety Agency (EASA).They are required to identify correctly 9 of the first 15 plates of the Ishihara (IH)24‐plates.The aim of this study was to assess the severity of CV loss of applicants in relation to certification outcome based on the EASA cabin crew protocol. 126 applicants (78 deutans, 46 protans and 2 normal trichromats) were tested.The IH, CAD(Colour Assessment and Diagnosis) and anomaloscope were carried out to the specification required for Class I certification. 72% (56/78) of deutans passed the IH, but only 37% (17 /46) of protans passed.For all subjects 40% (51/126) passed both the IH and CAD.8% (7 protans and 3 deutans) of subjects failed IH and passed the CAD.19% (23 deutans and 1 protan) passed the IH and failed the CAD and 7 of these subjects had severe CV loss on the CAD and unusual anomaloscope results. The IH is used widely in aviation, and the number of errors is often used as a measure of the severity of CV loss. This study shows that the number of errors is not a good indicator of the applicant’s severity of CV loss. When the current EASA protocol for cabin crewmembers based on IH is applied, severe colour deficient subjects can pass and less severe subjects fail. A revision of the current colour assessment protocol for the cabin crew is needed to produce justifiable minimum CV requirements using colour assessment tests that are less variable.
The role of Wnt/β‐catenin signaling pathway in lens and retina development
Authors: Kozmik Zbynek 
Published: December 2019

AbstractWnt/β‐catenin signaling pathway functions by regulating the amount of the transcriptional co‐activator β‐catenin that controls key developmental gene expression programs. However, β‐catenin is known to have a dual function in cells. Apart from its key role as a nuclear effector of Wnt/β‐catenin signaling pathway (transcriptional function), β‐catenin is an integral structural component of cadherin‐based adherens junctions (cytoskeletal function). The essential role of β‐catenin during development is demonstrated by the finding that β‐catenin knockout mice fail to develop beyond embryonic day E6.5. Tissue‐specific deletion of β‐catenin gene in lens and neural retina resulted in a number of ocular phenotypes such as formation of ectopic lenses, failure to form lens vesicle, altered fiber cell differentiation and disruption of typical laminated structure of neural retina. In order to distinguish the phenotypes resulting from cytoskeletal or transcriptional function of β‐catenin we used mouse strain, in which the endogenous β‐catenin is replaced by mutant form of the protein, retaining cytoskeletal but lacking transcriptional function. We found that mice deficient for transcriptionally competent β‐catenin manifest abnormal development of lens epithelium and ciliary marginal zone of the neural retina. Our data unequivocally identify specific roles of canonical Wnt signaling for lens and neural retina development.
Histological aspects of limbal stem cell transplantation
Authors: Gießl Andreas 
Published: December 2019

AbstractMaintenance and regeneration of the corneal epithelium relies on unipotent progenitor cells at the corneoscleral limbus, which are regulated by extrinsic factors from their local microenvironment, the stem cell niche. The postulated limbal niche is an anatomically protected site of intimate epithelial‐mesenchymal interaction and is highly vascularised, innervated, pigmented due to intraepithelial melanocytes. In addition, the limbal niche is infiltrated with immune cells, and supported by a specialized extracellular matrix as well as subepithelial mesenchymal cells emitting soluble signals. For ex vivo expansion and transplantation, limbal stem cells are unfavourably removed from their niche. This lecture outlines our current understanding and novel findings regarding the structural and molecular composition of the limbal niche including specific matrix components, cell‐matrix‐ and cell‐cell adhesion molecules, and niche cell populations, which are involved in stem cell regulation through multiple signalling pathways. This lecture also provides an overview of current tissue‐engineering approaches for corneal surface regeneration that aim at incorporating specific niche components, such as matrix proteins, growth and signalling factors, or putative niche cells, into the culture systems in order to support maintenance of stemness and to improve the therapeutic use of limbal stem cell transplantation.
Outcome of DMEK with prestripped grafts
Authors: Maier Philip 
Published: December 2019

AbstractDMEK has become the standard procedure for patients with Fuchs endothelial dystrophy. However, graft preparation seems to be more difficult than graft preparation for DSAEK. Therefore, more and more surgeons ask their eye bank for prestripped tissue for DMEK that can directly be used by the surgeon. In our hands clinical results following DMEK using surgeon stripped tissue were better than after DMEK using precut tissue. Yet, data from the literature show that using prestripped tissue compared to surgeon stripped tissue for DMEK can lead to equal clinical results. However, the kind of storage following preparation seems to be an important factor. In the talk we discuss advantages and disadvantages of prestripped tissue compared to surgeon stripped tissue used for DMEK with respect to the preparation and storage techniques.
ANCA‐associated optic neuropathy
Authors: Kawasaki Aki 
Published: December 2019

AbstractAntineutrophil autoantibodies (ANCAs) directed against either antiproteinase3 (anti‐PR3) or myeloperoxidase (anti‐MPO) are associated with a small vessel vasculitis in which the characteristic feature is a focal necrotizing damage of capillaries, venules and arterioles. The ANCA‐associated vasculitic syndromes are; granulomatosis with polyangiitis (formerly know as Wegener’s granulomatosis), microscopic polyangiitis, Churg‐Strauss syndrome and idiopathic necrotizing crescentic glomerulonephritis. Ocular and orbital manifestations may be a presenting manifestation of Wegener’s granulomatosis, in particular optic perineuritis. This presentation will review ANCA‐associated optic perineuritis and how it is distinguished from idiopathic optic perineuritis.
Post‐keratoplasty glaucoma; current state of diagnosis and treatment
Authors: Ratnarajan Gokulan 
Published: December 2019

AbstractIn this talk the examination and investigation used in the diagnosis of Post‐keratoplasty glaucoma will be considered, as well as potential challenges. The treatment options will be evaluated including traditional glaucoma surgery as well as newer treatment options such as sub‐conjunctival mircoshunts such as the Innfocus Microshunt and Xen Implant. Potential intra‐operative and post‐operative complications will be discussed as well as tips to minimise these.
CLET – Cultivated Limbal Epithelium Transplantation
Authors: Dobrowolski Dariusz 
Published: December 2019

AbstractInjury and inflammatory factors disturb the homeostasis of corneal epithelium renewal. The idea for restoring the physiologic condition is therefore not only to provide the proper cell population within a zone of damage, but also to ensure stable conditions of the surrounding microenvironment. Transplantation methods based on either allogeneic grafts or autologous corneal epithelial stem cells are recognized as effective methods of treating the limbal stem cells insufficiency. Treating the disease, we try to create optimal conditions for development and maintenance of the function of stem cells. Autologous approach, in recent years, has become an minimally invasive and the most efficient technique of treatment. Long‐term observations of such patients allow to present conclusions regarding presurgical as well as postsurgical management.
Clinical translation of eye drops and complex drug products
Authors: Kompella Uday B. 
Published: December 2019

AbstractEye drops constitute majority of ophthalmic drug products, with solutions, suspensions, ointments, and emulsions being the top formulations. While solutions are considered simple formulations, with biowaiver granted for qualitatively and quantitatively similar generic formulations, others are complex formulations, whose generic products are difficult to develop and translate. In particular, manufacturing processes as well as the grades of inactive ingredients used can affect drug product attributes and bioavailability. Thus, bioequivalence of complex generics is less certain. The US FDA issued some draft guidance documents to ease approval of complex generic drug products. One purpose of this presentation is to discuss the difficulties in clinical translation of complex generic eye drop products. In recent years, there has been significant effort in developing eye drops for back of the eye drug delivery, using either pure drug formulations or carrier‐drug composite formulations (e.g., polymeric nanoparticles), with little or no success to date. The second purpose of this presentation is to discuss translational gaps in advancing eye drops for back of the eye drug delivery and to further discuss the difficulties in manufacturing and stabilizing composite nanoparticle formulations for eye drop applications.
Non‐OPA1 genetic causes of dominant optic atrophies
Authors: Wissinger Bernd 
Published: December 2019

AbstractDominant optic atrophy (DOA) is one of the most common forms of inherited optic nerve disease. The prevalence of DOA has been estimated to range from 1:16 000–1:50 000 in different populations. Clinical expression of DOA is highly variable and reduced penetrance is rather common which may hamper to establish the proper mode of inheritance. Dominant optic atrophy is a genetically heterogeneous condition with mutations in the OPA1 gene being by far the most common cause of DOA. Yet the knowledge about other genetic causes of DOA is still limited and sometimes equivocally. In recent years it became apparent that ‐ in term of its genetic basis ‐ DOA is just part of a continuous spectrum of syndromic neurodegenerative disease in many of which optic atrophy is only one clinical feature. Notably, the vast majority of the involved genes encode for proteins which are imported into mitochondria and exert diverse biological functions therein. In this presentation, I will give an overview on the current knowledge of non‐OPA1 genetic causes of dominant optic atrophies and the potential molecular mechanisms underlying the clinical outcome as DOA or syndromic neurodegenerative disease.