bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric OVCR what’s new?
Authors: Bremond‐Gignac Dominique 
Published: December 2019

AbstractCentral retinal vein occlusion (CRVO) is the second cause of visual loss due to vascular disease after diabetic retinopathy. Its pathogenesis remains unclear but risk factors such as hypertension, glaucoma and age have been recognized. Indeed, CRVO occurs mainly after 50 years, and only 10–15% of patients are under 40 years of age. CRVO has been rarely reported in children, but often associated with a local cause (compressive or inflammatory), or a systemic condition as thrombophilic and hyperviscosity syndromes. The clinical presentation includes visual loss and the fundus shows optic disc edema, tortuous dilated retinal veins, retinal hemorrhages and signs of neovascularization at various levels as in adult cases. In children, the role of genetic variants of thrombophilia in the occurrence of CRVO remains unclear. We report 4 cases of central retinal vein occlusion (CRVO) in healthy children showing combined genetic variant of thrombophilia. Mutation of MTHFR (C677T) and GPIIIa PlA1/A2 seems to demonstrate implication in CRVO in children. In those cases, multidisciplinary consultation staff has to discuss preventive low‐dose aspirin therapy.
Modeling of Aniridia‐related keratopathy and identification of novel drugs for potential treatment
Authors: Aberdam Daniel 
Published: December 2019

AbstractHaploinsufficiency of PAX6 in humans is the main cause of congenital aniridia, a rare eye disease characterized by iris hypoplasia and reduced visual acuity. Patients have also progressive disorders including cataract, glaucoma and corneal abnormalities making their condition very challenging to manage. Aniridia‐related keratopathy (ARK), caused by a combination of factors including limbal stem‐cell deficiency, impaired healing response, abnormal differentiation, and infiltration of conjunctival cells onto the corneal surface, affects up to 95% of patients. It usually begins in the first decade of life resulting in recurrent corneal erosions, sub‐epithelial fibrosis with corneal decompensation and opacification. Unfortunately, current treatment options for aniridia patients are currently limited. Although animal models partially recapitulate this disease, there is no in vitro cellular model of AKT needed for drug/therapeutic tools screening and validation. We used genome editing to introduce a nonsense mutation into one allele of the PAX6 gene in limbal cells, a reservoir of stem cells able to differentiate into corneal cells. The mutated cells displayed reduced cell proliferation and cell migration but enhanced cell adhesion. Known PAX6 targets expression was also altered. By High Throughput Screening of a FDA‐approved library, we identified two small compounds that rescued both PAX6 endogenous protein level and altered cell migration of the mutant limbal stem cells. Since these two compounds are already used in psychiatric medicine, their repurposed use to treat topically aniridia eye could become realistic as soon as validated in vivo for efficacy and safety on aniridia‐like mice.
Insights into the Farnsworth D15 test and its efficacy in occupational protocols
Authors: Evans Benjamin 
Published: December 2019

AbstractSince their introduction, occupational colour vision (CV) standards have, in many ways, driven the development of CV tests. One test designed for occupational use is the Farnsworth D15 and despite having been introduced over 70 years ago, this test continues to be used to determine occupational suitability. To complete the test subjects are required to place 15 caps in order so as to minimise perceived differences between adjacent caps when illuminated with daylight (D65). Both red‐green (RG), yellow‐blue (YB) colour and luminance signals can contribute to what a subject perceives as a minimum difference between adjacent caps. The D15 aims to fail all monochromats, dichromats and subjects with severe anomalous trichromacy whilst passing all normal trichromats and only the least severe subjects with colour vision deficiency (CVD). The purpose of this study was to investigate the D15 test and its efficacy in occupational protocols. The different signals rod monochromats, dichromats and subjects with normal CV make use of most when presented with the D15 test were established, along with reliable D15 statistics for normal, deutan and protan subjects. A model was also developed to predict the colour signals involved, and the expected cap orders generated when subjects with a range of CVD carry out the test. When no crossings and up to two adjacent transpositions are allowed on the D15 test, 100% of normal trichromats (N = 95), 56% of deutans (N = 325) and 47% of protans (N = 170) pass. 43% of protans and 23% of the deutans that pass have RG thresholds above 10 CAD units (one CAD unit describes the mean RG colour signal strength for young normal trichromats). As one would expect the pass rate can vary significantly under different occupational protocols. As predicted by the model, rod monochromats, deuteranopes and protanopes were able to complete the Farnsworth D15 test when the caps were separated into two groups, despite severe loss or even complete absence of colour vision.
Colour assessment in patients at risk of developing diabetes
Authors: Rodriguez‐Carmona Marisa 
Published: December 2019

AbstractDiabetes has frequently been linked to loss of colour vision. A recent study has shown that over 70% of diabetics have significant loss of both Yellow/Blue (YB) and Red/Green (RG) colour vision (https://doi.org/10.1111/j.1755-3768.2012.f073.x), in the absence of clinical signs of retinopathy. This study also showed poor correlation between the severity of RG or YB loss and parameters such as duration of diabetes, age, HbA1c, body mass index, visual acuity or central retinal thickness. The aim of this study was to investigate whether clinically ‘normal’ subjects, who have been identified as being at ‘risk’ of developing diabetes, show significant changes in colour vision and rod and cone sensitivities.A ‘high‐risk’ group (n = 62) and subjects diagnosed with diabetes (n = 23) were recruited from a healthcare centre that offers diagnostic and screening services, including vision assessment. The inclusion criteria for ‘high‐risk’ group required 3 or more recognised risk factors for diabetes (e.g., age >45, HbA1C >5.7, high blood pressure, smoking history, high BMI, family history of diabetes, FPG levels >100 mg/dl). All subjects had full ophthalmic assessment and their RG and YB colour vision, Functional Contrast Sensitivity, Visual Acuity and Rod‐ and Cone‐mediated flicker sensitivity were measured using the Advanced Vision and Optometric Tests (AVOT) developed at City University (www.avot/city.ac.uk).Consistent with previous findings, the diabetic group show significant loss of both RG and YB colour vision. They also have higher thresholds for rod‐ and cone‐mediated vision. Surprisingly, the ‘high‐risk’ subject group who do not meet the clinical criteria for early diabetes also show significant loss of colour and rod and cone sensitivity. These findings suggest that accurate assessment of colour vision and rod and cone mediated thresholds can be used as early biomarkers in in prediabetic screening.
Megalocornea in the Czech Republic‐additional clinical features and novel disease‐causing variants
Authors: Liskova Petra 
Published: December 2019

AbstractX‐linked megalocornea is a congenital disorder, characterised by bilaterally enlarged corneas, with a horizontal white‐to‐white measurement of ≥13 mm, reduced central corneal thickness, and deep anterior chamber, without increase of the intraocular pressure. The results of clinical examination and mutational analysis in four families with the occurrence of megalocornea will be presented, including previously unreported phenotypic features and novel CHRDL1 mutations.
Mini‐DMEK for focal defects of Descemet's membrane
Authors: Bachmann Björn 
Published: December 2019

AbstractIntroductionDescemet Membrane Endothelial Keratoplasty (DMEK) has evolved into a standard procedure for patients with corneal endothelial diseases. Recent reports suggest that DMEK can also improve corneal edema in patients with breaks in Descemet's membrane (DM) as in acute hydrops.MethodsThis is a retrospective analysis of consecutive patients after DMEK with small grafts (Mini‐DMEK) for the treatment of defects of DM. The size of the graft in Mini‐DMEK was adapted to the size of the defect of DM.ResultsFour out of six Mini‐DMEKs were performed in patients with acute hydrops in keratoconus. One Mini‐DMEK was performed in a patient with a large central tear after DALK and one Mini‐DMEK was performed in a patient with Haab's striae in primary congenital glaucoma. Incomplete graft attachment necessitated one re‐Mini‐DMEK in a patient with acute keratoconus. Intraoperatively, unfolding and positioning of small grafts turned out to be more complex than in DMEK with grafts of normal size (≥7 mm). After successful Mini‐DMEK corneal thickness and corneal edema decreased in all patients.ConclusionMini‐DMEK is a viable treatment option for patients with ruptures or breaks of DM. Future analysis are needed for better identification of patients with defects of DM who will benefit from Mini‐DMEK.
Combined surface ablation+CXL for keratoconus
Authors: Balidis Miltos 
Published: December 2019

AbstractKeratoconus treatment should be aimed to improve the optical inefficiency of the irregular cornea and halt the disease progression that may lead to the need for corneal transplantation. Corneal collagen cross‐linking (CXL) using Riboflavin and ultraviolet A has been used to stabilize the cornea with progressive keratoconus or ectatic corneal disorders after corneal refractive procedures. Studies demonstrated that the simultaneous wavefront optimized (WG PRK) or topography guided (TG PRK) approach of photore‐fractive keratectomy (PRK) plus CXL offers improvements in uncorrected distance acuity (UDA), best distance acuity(BDA), and topographic irregularity, even if the surgical goal is not a refractive end point. Combining photorefractive keratectomy (PRK) with CXL or accelerated CXL offers keratoconus patients both stability and improvement in functional vision. However, different excimer laser platforms have different ablation patterns and different algorithms for topography‐guided ablation. Additionally, mechanical and laser debridement of the corneal epithelium (PRK versus transepithelial PRK) may result in different patterns of ablation and different clinical results. Though the conventional refractive components of sphere and cylinder may be simultaneously incorporated into the treatment profile, in the treatment of keratoconus, tissue sparing is the prime consideration, and therefore, PRK typically limits the maximum depth of tissue ablation to less than 50mm. Several CXL protocols result in more cross‐linking and a greater increase in Young's modulus, less corneal cross‐linking (a smaller increase in Young's modulus) may still have the same clinical effect (in terms of disease stabilization). In other words, the optimum CXL parameters to produce the clinically desired effect (in terms of stabilization or prophylaxis) are not known. A reduction in HOA after TG‐PRK in keratoconus eyes (with or without the addition of CXL) is not surprising and is a consistent finding in published studies. Diffuse corneal haze, which emerges in a few days after CXL in virtually all eyes, generally resolves over time. It is identified as a dust‐like change in the stroma. In addition, some patients suffer from scarring that persists and can decrease visual acuity. However, these two words— “haze” and “scar”—are sometimes used interchangeably in the literature.
Neurotrophic keratitis as a peripheral sensory neurodegenerative disease
Authors: Aracil Adolfo 
Published: December 2019

AbstractMaintenance of the ocular surface critically depends on the nocifensive responses elicited by the proper functioning of corneal sensory innervation. Damage of any type caused to the trigeminal ganglion or its ophthalmic branch is associated to the development of chronic trophic disturbances of the corneal epithelium homeostasis, which may progress to corneal perforation and vision loss. Since the first observations by Magendie as early as in 1824, many efforts have been dedicated to identifying the molecular mechanisms underlying the trophic effects exerted by sensory neurons on corneal tissue.Many molecules derived or released by sensory nerves have been involved in corneal epithelial wound healing, either in vivo or in vitro. Neuropeptides released from the intraepithelial free nerve endings of corneal polymodal nociceptors have been claimed as potential candidate molecules to explain the trophic effects of sensory neurons on the corneal epithelium. However, their precise role in basal maintenance of the corneal epithelium or during corneal wound healing is far from being clearly understood. Consequently, neurotrophic keratitis is still considered as an orphan disease, and its association to the trigeminal sensory neuron degeneration is the rationale for assaying neuroregenerative therapies, such as neurotrophin eyedrops or neurotization, as potential treatments.(Supported by grants SAF‐2017‐83674‐C2‐1‐R and 2‐R from Agencia Estatal de Investigación, Spain, and ERDF, EU, and PROMETEO/2018/114 from Generalitat Valenciana, and in part by Horizon 2020 G.A. No 667400, European Commission)
Intracorneal Ring Segments in Keratoconus; an Evidenced‐based Approach
Authors: Gatzioufas Zisis 
Published: December 2019

AbstractImplantation of intracorneal ring segments (ICRS) is an effective and safe surgical option for visual improvement in keratoconus. ICRS are small, PMMA devices, which are implanted into the cornea aiming to alter its geometry in a manner that it will enhance its refractive properties and thereby will improve visual acuity. Their implantation was initially managed mechanically, but over the last years femtosecond laser‐assisted implantation has been gaining increasing interest and is gradually replacing the conventional mechanical technique. Nowadays, there are implantation nomograms indicating the appropriate ring segment characteristics for each individual case and suggesting, in a customized manner, the most suitable parameters for ICRS implantation. There are also certain indications and contraindications regarding the patient selection in order to maximize the safety and efficacy of the treatment. The purpose of this presentation is to update the current status of ICRS implantation as a modern therapeutic option in the visual rehabilitation of patients with keratoconus, highlighting pearls and pitfalls for both young and experienced corneal surgeons.
Protective Role of Xtra‐Factor in Refractive Surgery
Authors: Gatzioufas Zisis 
Published: December 2019

AbstractCombination of refractive surgery procedures (i.e. LASIK) with high‐fluence, accelerated corneal crosslinking (Xtra) has been proposed and introduced in clinical practice, aiming to restore corneal strength, increase stability of visual outcomes, and potentially lower the refractive enhancement rate. However, there is no consensus in the literature regarding the beneficiary effect of this procedure.This presentation will review current clinical evidence on the topic, highlight advantages and disadvantages of this method and, finally, deliver relevant practical tips for young corneal surgeons.
The AkkoLens Lumina accommodative intraocular lens
Authors: Rombach Michiel 
Published: December 2019

AbstractThe AkkoLens Lumina is an accommodative intraocular lens based on cubic, Alvarez, optical elements. The Lumina is designed to be implanted into the ciliary plane with the Lumina customized for the individual eye with each eye having its specific combination of refraction and sizing. The lens provides up to 4D of accommodation and provides a visual acuity and contrast sensitivity similar to a monofocal lens. The current state of development of the Lumina and clinical results are presented.
Anti‐AQP4 and anti‐MOG optic neuropathies
Authors: Yu‐Wai‐Man Patrick 
Published: December 2019

AbstractNeuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system (CNS) that preferentially targets the optic nerve and the spinal cord. Due to the significant risk of permanent visual and neurological impairment in NMO, rapid diagnosis and the prompt initiation of the most appropriate treatment are essential. Antibodies against the aquaporin‐4 (AQP4) water channel and myelin oligodendrocyte glycoprotein (MOG) have been identified in patients with NMO phenotypes. In this presentation, the clinical and neuropathological features of anti‐AQP4 and anti‐MOG optic neuropathies will be reviewed with a critical appraisal of current treatment options.
Glaucoma Surgery‐ How to manage the failing trabeculectomy
Authors: Crawley Laura 
Published: December 2019

AbstractIn this session we will explore the short and longer term strategies for maximizing filtering bleb surgery success. This will include pre, intra and post operative management tips. We will explore the common and unusual reasons for bleb failure and ask the question is there a place for redo trabeculectomy in the modern era of glaucoma drainage devices.
How does aging influence tear fluid proteomics
Authors: Nättinen Janika 
Published: December 2019

AbstractThe prevalence of many ophthalmic diseases increases with age. While the clinical characteristics and pathophysiological mechanisms of these diseases are relatively well‐known or extensively studied, the underlying biological systems of normal aging in the ocular surface remain unclear. In order to examine the effects of aging on tear fluid proteomics and ocular surface, tear microcapillary samples were collected preoperatively from 115 subjects and analysed using NanoLC‐TripleTOF mass spectrometer. Bioinformatics analysis revealed several inflammation and immune response‐related proteins, which correlated significantly with increased age. Our results display the effects of normal aging in the ocular surface and identify a group of age‐dependent proteins. Knowledge of the proteins, pathways and upstream regulators discovered in our study can be later implemented in the research of dry eye and other ocular surface diseases, which are associated with increased age.
Diagnosis of haematological diseases with monoclonal gammopathy
Authors: Mikala Gabor 
Published: December 2019

AbstractDespite the complexity of lymphoproliferative disorders, the presence of monoclonal immunoglobulins has been providing diagnostic guidance to the haematologist for more than a century. Clinicians may be alerted for the possible presence of serum monoclonal immunoglobulins produced in excess by increased erythrocyte sedimentation rate. Thereafter the diagnosis of monoclonal gammopathy could be proven by serum protein electrophoresis followed by immunofixation to properly identify the M‐protein. In a quarter of the cases, no intact monoclonal immunoglobulin could be detected in the sera, however, free light chains produced in excess may be identified using specialized immunoassays, so called free light chain analysis.Idiopathic monoclonal gammopathy of unknown significance (MGUS) may be present in 1% of the population above age 50 and up to 10% for individuals over 80 years of age. Due to the high frequency of MGUS, between 50‐65% of all monoclonal proteins detected fall into this category and most asymptomatic patients remain undetected for many years. MGUS patients have a 1% per year chance for transformation into malignant monoclonal gammopathies. Presently, it is accepted that most multiple myeloma and amyloidosis patients have their disease developed by transformation from preexisting MGUS. Additionally, some nonmalignant hematological disease also frequently exhibit monoclonal immunoglobulin synthesis, most notably Gaucher’s disease.From the field of neoplastic hematological diseases, smouldering and symptomatic multiple myeloma as well as Waldenström’s macroglobulinaemia almost always exhibit significant amounts of serum M‐protein and/or monoclonal free light chain excess. In case of solitary plasmocytoma, primary AL amyloidosis, cryoglobulinaemia and plasma cell leukemia, the correlation with gammopathy is less strict. Monoclonal gammopathy is less common in different types of non‐Hodgkin lymphoma. Within lymphomas lymphoplasmocytic lymphoma and marginal zone lymphoma more commonly secrete monoclonal immunoglobulins, while other B‐cell lymphomas have 14% cumulative chance for specific gammopathy. In the case of B‐cell chronic lymphocytic leukemia (the most common leukemia), intact monoclonal gammopathy has a 2% appearance, while abnormal free light chains may be detected in a third of the cases. Sporadically monoclonal gammopathy may be detected in chronic myelomonocytic leukemia, refractory anaemia, large granular cell leukemia and angioimmunoblastic lymphoma. Notably, in many of these diseases, the amount of secreted monoclonal immunoglobulin and/or free light chains correlate with the tumor burden and could be used for disease monitoring, especially in indolent hematological neoplasias.
Small graft, simultaneous amniotic membrane transplantation, temporary lateral tarsorrhaphy and autologous serum improves outcome of penetrating keratoplasty in congenital aniridia with aniridia associated keratopathy
Authors: Fries Fabian 
Published: December 2019

AbstractPurposeCongenital aniridia is a challenge not only for the corneal surgeon. Persisting epithelial defects, suture loosening, and an increased risk of graft rejection are typical postoperative complications in high risk keratoplasty such as in congenital aniridia.MethodsBetween 2012 and 2018, 20 keratoplasties were performed in 13 patients with congenital aniridia at our Department. Visual acuity and intraocular pressure before and after keratoplasty, corneal graft size, suture technique (interrupted or double running), use of amniotic membrane transplantation and serum eye drops, size of bandage contact lens as well as morphological and functional status during follow‐up were analysed retrospectively.ResultsSix keratoplasties were performed using continuous double running suture, 14 using interrupted sutures. Amnion membrane transplantation as a patch with a 17 mm bandage contact lens was applied 18 times to promote postoperative corneal wound healing. In 10 cases, a simultaneous temporary lateral tarsorrhaphy was performed to support corneal wound closure. All patients received systemic immunosuppression. Visual acuity improved in 14 eyes at 6 months follow‐up, in 6 eyes visual acuity remained stable, no eye showed a decrease in visual acuity.ConclusionsKeratoplasty in congenital aniridia must be considered a high risk keratoplasty. A modified keratoplasty scheme combining a small corneal graft size, interrupted sutures, simultaneous AMT Patch, large bandage contact lens, temporary lateral tarsorrhaphy, postoperative autologous serum eye drops, and systemic immunosuppression seems to be appropriate to reduce the postoperative complication rate in these most difficult eyes.