bluuebunny/crossref_metadata_2025_split huggingface dataset:
New concepts of treatment for LHON and other genetic mitochondrial optic neuropathies
Authors: SADUN A 
Published: November 2013

AbstractIn Leber’s Hereditary Optic Neuropathy (LHON), complex I impairment can lead to subacute loss of vision and optic nerve atrophy, usually in young adulthood. This blockage of electron transfer produces some decrease in ATP production, but also a severe accumulation of reactive oxygen species (ROS) that is thought, at a critical threshold, to lead to RGC apoptosis. Co‐enzyme Q10, a quinone, has the potential to reroute the spilled electron, however treatment with co‐enzyme Q10 has been ineffective. Idebenone, a second generation quinone with better drug delivery characteristics, has demonstrated some efficacy in LHON (results presented in a companion presentation). EPI‐743 is a third generation quinone that has other promising characteristics. We provided EPI‐743 (300 mg, TID) in an open label trial to 12 patients with recent visual loss from LHON and carefully followed 23 eyes for 2‐3 years (16 to 40 months). Seven of 23 eyes demonstrated improvements in visual acuity and 5/23 improvements in HVF. Thirteen of 23 eyes showed improvements or at least acute stabilization of vision with these outcome measures. Oddly, the time to recovery was very long (9 to 15 months).
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The fate of the corneal endothelium during storage: apoptosis/necrosis/survival
Authors: ARMITAGE J 
Published: November 2013

AbstractCell death during corneal storage is, at least with current methods, inevitable. There are also concerns with a tissue such as cornea for the maintenance of cell‐cell and cell‐matrix interactions that are so vital for overall physiological function of the tissue. The reversibility of such changes is clearly of fundamental importance. However, once cells are committed down a pathway of cell death, there is perhaps little that can be done to reverse this process. The terminology applied to describe cell death perhaps clouds our perception and too easily leads us to categorize cell death simply as apoptosis or necrosis. In particular, the term necrosis merely describes an end point and sheds no light on the mechanism by which a cell died, for example by oncosis. Understanding the mechanisms and pathways to cell death may, however, provide insights into novel targets for supporting cells, reducing cell loss, and improving functional integrity.
Evaluation of the donor tissue during storage
Authors: BORDERIE V 
Published: November 2013

AbstractDevelopment of new surgical techniques for corneal transplantation induces major modifications in the graft selection process in eye banks. Quality of the donor corneal tissue must currently be assessed according to the scheduled surgical procedure. Not only the endothelial layer should be assessed by conventional techniques (trypan blue staining, light microscopy, specular microscopy) but also the corneal stroma and epithelium. This latter task requires development of new techniques such as high definition optical coherence tomography. Full field‐OCT, in providing a detailed study of corneal structures with ultrahigh resolution (1 µm in all directions), permits detecting corneal conditions that are currently difficult to identify in eye banks, such as keratoconus, absence of Bowman’s layer, or corneal scars. It permits evaluating precisely Descemet’s membrane condition. It seems to be a promising technique in complement to current assessment methods of human donor corneas. Proper evaluation of the donor tissue by the new technologies implies formation of the eye bank staff. The staff has to get close relationships with the surgeons and to be attentive to the special requirements related to the scheduled surgical technique.
Preparing donor tissue for DSEK
Authors: DICKMAN M 
Published: November 2013

AbstractDonor tissue preparation remains a critical step in endothelial keratoplasty technique. Since its introduction in 2008 the use of pre‐cut grafts for Descemet stripping automated endothelial keratoplasty (DSAEK) has become increasingly popular. Pre‐cut tissue enables additional quality control, not readily available in the operating theater, shortens operation time, eliminates intraoperative donor related complications, overcomes surgeon’s learning curve associated with valuable donor tissue loss and allows surgeons who do not have the necessary equipment to perform the procedure. Previous reports suggesting thinner grafts may result in better visual outcomes have driven surgeons to either try and harvest ultra‐thin grafts themselves or request eye banks to do so for them. However, preparation of ultra‐thin grafts is not without challenges. In the current session the literature on the safety and efficacy of precut tissue for (ultra‐thin) DSAEK will be reviewed. The results of our in‐vitro studies on thickness reproducibility and endothelial cell viability of ultrathin precut tissue for DSAEK, using an innovative microkeratome system, as well as our preliminary clinical experience with these grafts will be presented.
Controversies in the pathogenesis of RVO – Systemic perspective
Authors: GRZYBOWSKI A 
Published: November 2013

AbstractAlthough retinal vein occlusion (RVO) has been known for about 150 years, and traditionally was assumed to be one entity, there are many arguments that RVO actually is not one disease. It has been suggested that it actually consists of six different clinical entities, each with different clinical characteristics, prognosis and management. For example, central retinal vein occlusion (CRVO) etiologically, clinically and from the management point of view is totally different from branch retinal vein occlusion (BRVO), and that is also true in the case of the major and macular retinal vein occlusions. Similarly, it is well‐established now that CRVO is of two types, ischemic and non‐ischemic, with very different clinical characteristics and management. It has also been shown that pre‐existing ocular hypertension or glaucoma are important risk factors for CRVO and hemi‐central retinal vein occlusion but not for BRVO. Many risk factors indicate systemic nature of some forms of the disease, although their real value remains speculative. Therefore, the lumping them together as one disease might be incorrect and misleading. Taking all above into account the traditional single disease model of RVO should be re‐evaluated.
Anti‐VEGF agents in the treatment of RVO
Authors: REHAK MATUS 
Published: November 2013

AbstractVascular endothelial growth factor (VEGF) is a key player in the development of macular edema (ME) in patients with retinal vein occlusion (RVO). However various other factors influence the development of ME. In a rat model, we observed a fast and transient upregulation of the expression of VEGF, which is dominant for the acute stage of ME. In chronic ME an upregulation of inflammatory factors is presented. The intravitreal drugs reduce the ME, but the mechanism of action differs between steroids and anti‐VEGF. Anti‐VEGF drugs inhibit the VEGF whereas steroids presents an unspecific anti‐inflammatory effect inhibiting the expression of broad spectrum of molecules. The determination of the patients who benefit from each particular treatment would improve the functional results and reduce the possible side effects. In a retrospective study in which we investigated whether BRVO patients benefit more from injection of bevacizumab or triamcinolone if treated according to the duration of ME we found out a better visual gain under treatment with bevacizumab in the first 4 months. In patients with treatment later than 4 months since onset of BRVO no significant differences between bevacizumab and triamcinolone were observed.
Cognitive function associated with larger optic nerve heads
Authors: JONAS J , XU L , WANG YX , LI JJ 
Published: November 2013

AbstractPurpose To assess associations between optic nerve head size and parameters of cognitive function in a population‐based study design.Methods The Beijing Eye Study is a population‐based cohort study in Northern China and included 4442 subjects. The study participants underwent a detailed ophthalmologic examination and an interview with questions on the level of education. Using fundus photographs, we measured the size of the optic disc.Results Assessable optic disc photographs were available for 4089 (92.1%) subjects. After adjusting for age, gender, refractive error, and best corrected visual acuity, optic disc size was significantly associated with a higher level of education (P<0.001) and shorter time needed to perform frequency doubling threshold perimetry (P<0.001). In a reverse manner, the level of education was significantly associated with increasing optic disc size (P<0.001) and shorter perimetric test duration (P<0.001) after adjustment for age, gender and best corrected visual acuity.Conclusion In a population‐based study on adult Chinese, optic nerve head size was associated with the highest achieved level of education and time needed to perform a standardized perimetric test. Considering level of education and time needed to perform perimetry as surrogates of cognitive function, optic nerve head size was related to cognitive function.
Anterior pathway meningiomas
Authors: KAWASAKI A , BOSCHI A 
Published: November 2013

AbstractMeningiomas can cause visual disturbance by interrupting the afferent or efferent pathway of vision. Optic nerve sheath and clinoid meningiomas cause optic neuropathy, cavernous sinus meningiomas cause ocular motor palsies and occipital meningiomas cause homonymous hemianopsias. Case examples , pearls for radiographic detection and issues related to management will be discussed
The eye screen of the German Mouse Clinic (GMC) ‐ New genetic insights into eye development and ocular disorders
Authors: PUK O , FUCHS H , GAILUS‐DURNER V , HRABE DE ANGELIS M , GRAW J 
Published: November 2013

AbstractPurpose To summarize GMC ‐ Eye Screen findings with about 350 tested mouse mutant lines.Methods Eye morphology was analyzed by Slit Lamp Biomicroscopy/Scheimpflug Imaging and Ophthalmoscopy/Optical Coherence Tomography, eye size by Laser Interference Biometry, and the Virtual Optokinetic Drum was used for testing visual ability.Results The GMC is a large‐scale phenotyping center where mouse models for human diseases are analyzed in a standardized way. More than 550 parameters are investigated in 14 different areas including behavior, neurology, cardiovascular functionality and vision and eye development. In the past 12 years, we identified eye pathologies in 43 mutant lines of the GMC workflow. The majority of them exhibited irregular eye sizes (72%), followed by morphological changes as corneal and lens opacities (21%) or retinal damages (16%) and moderately reduced visual abilities (7%). Overall phenotype predictions (EuroPhenome database; http://www.europhenome.org/) indicated that 89% of these lines were additionally affected in behavioral and/or neurological parameters, pointing to general relations between eye and brain development. Moreover, 46% showed heart impairments, connecting eye diseases with cardiovascular risks. Our data further proved for 11 gene products a previously unknown participation in ocular processes. These include the chromatin modifiers Asxl1 and Mysm1, the transcription factor Nfya as well as Arvcf, Jmjd5 and Slc20a2 with a role in cell‐cell communication, protein hydroxylation and phosphate transport, respectively.Conclusion The GMC ‐ Eye Screen is an effective approach for improving knowledge about ocular genetics and detecting associations with non‐ocular disorders.
Global microarray analysis and metabolic pathway profiling of a transgenic model of conditional, selective Müller cell ablation
Authors: CHUNG SH , SHEN W , YANG J , JAYAWARDANA K , GILLIES MC 
Published: November 2013

AbstractPurpose Müller cells, the principal glia cells in mammalian retina, play a critical role in retinal homeostasis. We have developed a transgenic Cre‐Lox model for selective, conditional Müller cell ablation to examine the relationship between Müller cell dysfunction and retinal diseases. The purpose of this study was to examine differentially expressed genes and their related pathways with microarray analysis, then to profile metabolic pathways in areas of Müller cell ablation.Methods Affymatrix microarray was performed on whole retina samples 1 week, 1 month and 3 months after induced Müller cell ablation. Data were analysed with limma package (p<0.05) and qRT‐PCR was used for array validation. Isolation of patches of Müller cell ablation was achieved by laser capture microdissection (LCM) and qRT‐PCR was conducted on pathway related genes. Immunofluorescence microscopy was used to validate results.Results Neuroprotection and apoptosis‐related genes were upregulated 1 week after Müller cell ablation, angiogenesis, tight junction and metabolic‐pathway related genes were downregulated later. Further analysis of glycolytic and mTOR pathways with tissue obtained by LCM revealed significant downregulation of genes related to these pathways in patches of Müller cell loss compared with controls. Immunofluorescent studies revealed that the dowregulations mainly occurred in the photoreceptor segments, although Enolase1 colocalised with Müller cells.Conclusion We found reduction of transcription and expression of proteins involved in key metabolic pathways in patches of Müller cell ablation. This study provides new insights into the relationship between Müller cell dysfunction and retinal diseases.
Wound healing in health and disease
Authors: UUSITALO H 
Published: November 2013

AbstractWound healing is a complex process involved in ocular surgery, trauma and pathogenesis of several eye diseases. Due to the delicate and sensitive structures of the eye wound healing is playing a essential role in ophthalmology. The transparency and optical properties of the cornea makes the mechanisms involved in the wound healing after the corneal surgery important understand and treat in detailed way. In glaucoma surgery well controlled wound healing process is as important for the creation of a functioning passage to aqueous humor out of the eye. The state of the ocular tissues is of great importance for the success of ocular surgeries and further points out the role of inflammation in wound healing processes. For example in case of glaucoma surgery the ultimate goal is to prevent ganglion cell death by lowering the intraocular pressure. Because ocular surfaces are the most important pathway for the topical glaucoma drugs and thus most vulnerable to the topical adverse reactions, they influence on the preoperative state of these tissues and thus the success of glaucoma surgery. The wound healing SIS is focusing in the mechanism involved in the wound healing in general and particularly in corneal and glaucoma surgery.
Changes in choroidal thickness in adult onset foveomacular vittelliform dystrophy versus exudative AMD
Authors: COSCAS F , PUCHE N , SOUIED E , COSCAS G 
Published: November 2013

AbstractTo compare choroidal thickness (CT) between eyes with adult onset foveo macular vitelliform dystrophy (AOFVD) eyes with age‐related macular degeneration (AMD). 5 group (38 eyes each) with AOFVD, fellow eyes with AOFVD wet AMD, dry AMD, age‐matched normal subjects.Choroidal thickness was measured using enhanced‐depth imaging optical coherence tomography (EDI‐OCT) with Spectralis. Subfoveal choroidal thickness (SFCT) in each eye was analyzed with measurement of the vertical distance from the Bruch’s membrane to the innermost choroid/sclera junction, at 500µm intervals up to 1500µm, from temporal to nasal and from superior to inferior to the center of the fovea.The difference between SFCT AOFVD (325.66 +/‐ 86 µm) versus CT in wet AMD (168.21 +/‐ 57.7 µm) and CT in dry AMD (158.77 +/‐ 68 µm) and versus elderly normal (238,33 +/‐ 83µm) are statistically significant, (p < <0.0001).The difference found between the CT of the affected eye and the fellow eye in AOFVD (325.66 +/‐ 86 µm) is not statistically significant (p = 0.6).This study demonstrates thickening of choroid in the eyes with AOFVD, in contrast with choroidal thinning observed in eyes with AMD. CT could become criteria for diagnosis and specific treatment AMD vs AOFVD
Variations of choroidal thickness in exsudative AMD before and after treatment
Authors: MAUGET‐FAYSSE M , COSCAS G 
Published: November 2013

AbstractPURPOSE: To evaluate choroidal morphological changes 3 and 6 months after intravitreal injection of Ranibizumab for exudative age related macular degeneration. METHODS: Retrospective, nonrandomized study, including patients with exudative AMD treated with Ranibizumab intravitreal injection and followed during 6 months. Non treated eye of AMD patients will be consider as control. All patients underwent a complete ophthalmological examination. At baseline, 3 months and 6 months, foveal and non‐subfoveal areas were evaluated with Spectralis optical coherence tomography on both eye: under the fovea and at 500 and 1500 μm nasally and temporaly . RESULTS and CONCLUSION: All studied eyes had exudative AMD at baseline treated with at least 3 Ranibizumab (Three Ranibizumab followed by injections if necessary were done for each patients). The results will be presented during the meeting.
Congenital hypertrophy of the RPE related to familial adenomatous polyposis
Authors: DESJARDINS L 
Published: November 2013

AbstractIntroduction: Familial adenomatous polyposis is a rare condition found in 1/10000 births. Multiple bowel polyps can lead to bowel cancer if untreated. Multiple pigmented retinal lesions are present in 60 to 80% of patients with this condition. Clinical presentation and imaging: Fundus lesions are classified in five groups: ovals pigmented with halo, small pigmented round , large pigmented round, depigmented round and small hypopigmented. They can be grouped with a bear track aspect. The lesions are asymptomatic A total number of at least four lesions is considered a good marker of the disease. Angiographic aspect is only mask or window depending on the degree of pigmentation. Pathology: Histologically the lesions are made of one or several layers of pigmented hypertrophic epithelial cells. Genetic: familial adenomatous polyposis is linked to a germinal mutation of the APC gene which is localized on the long arm of chromosome 5.
The role of in vitro studies in eye research
Authors: UUSITALO H 
Published: November 2013

AbstractIn vitro techniques are commonly used in eye research and they are valuable tools with several benefits like their modest price, well controlled test environment and the relatively simple interpretation of the results. They are after their validation usually relatively easily and quickly performed and in most cases suitable for automated analyses. In many cases their use can replace in vivo studies with experimental animals and thus they are ethically sustainable. By using valid in vitro techniques it is also possible to simplify the research frame from very complex in vitro situation and to set more specified hypothesis. There are, however, several drawbacks in the use of in vitro techniques. The results of them should be analyzed critically and the validity of the used in vitro technique should be rechecked on the basis of the new knowledge. It should also be kept in mind that an in vitro technique is usually valid for one specific cell mechanism and could be unacceptable for another.
Why is the optic nerve the canary in the coal mine of mitochondrial diseases?
Authors: SADUN A 
Published: November 2013

AbstractMitochondrial Optic Neuropathies (MON) present characteristic clinical and pathological features. Loss of visual acuity, dyschromatopsia and a central scotoma speak to involvement of the papillomacular bundle (PMB). Histopathology confirms this. These features are seen in hereditary diseases (Leber's Hereditary Optic Neuropathy—LHON and Dominant Optic Atrophy), Syndromes (such as MELAS, Wolfram's or FA), or toxic MONs. So why is the optic nerve involved so early and so often in mitochondrial disease? The brain weighs 2% of the body but consumes 20% of the body's oxygen. This expensive consumption of energy is largely due to the need for repolarization of the axon's membrane after each action potential. This is largely mitigated by myelin such that the sodium/potassium pumps only work at the Nodes of Ranvier. However, the retinal nerve fiber layer remains unmyelinated due to the need for inner retinal transparency. We present a mathematical model of these conditions that predicts the order of fiber loss in MON as a function of myelin and axon diameter. We demonstrate, with morphometry of postmortem samples of normal and LHON optic nerves, that this pattern is strictly observed.
Toward a new classification of RVOs?
Authors: PAQUES M 
Published: November 2013

AbstractThe proteiform features of RVO challenges classification. The discovery of patients with abrupt visual loss due to severe hypoperfusion, manifesting by perivenular whitening (PVW) in the absence of nonperfusion, raises the question of the pertinence of the ischemic versus nonischemic classification. High resolution OCT showed that focal ischemia in these cases was restricted to the inner nuclear layer, the atrophy of which accounted for clinical sequelaes. On the opposite, there are evidence that most cases of chronic macular edema are preceded by weeks or months of progressive venous dilation, hence raising the question of the true duration of the disease. Finally, we evidenced that venous macroaneurysms are a frequent consequence of longstanding RVO, whose identification by ICG angiography and subsequent photocoagulation may improve visual outcome. We will propose here a novel classification of retinal vein occlusions taking into account these features.
Reliability of the cone counts by an Adaptive Optics Retinal Camera
Authors: BIDAUT GARNIER M , FLORES M , TUMAHAI P , MEILLAT M , DEBELLEMANIèRE G , PUYRAVEAU M , SCHWARTZ C , MONTARD M , DELBOSC B , SALEH M 
Published: November 2013

AbstractPurpose To report the reproducibility and the repeatability of cone imaging in healthy human eyes, using the RTx‐1™ Adaptive Optics Retinal Camera, (Imagine Eyes, Orsay, France) and AOdetect v0.1 cone counting software.Methods Cones were imaged in healthy emmetropic aduts. Mean cone density (cells/mm2), spacing between cells (µ) and percentage of hexagonal cells calculated on Voronoi diagrams, were measured with the proprietary software of the device, from a 0.3°square, positioned on a horizontal line crossing the fovea. The square was moved horizontally following a 10µ step, until reaching the parafoveal density peak. Intersession reproducibility was assessed by imaging 3 subjects, 5 consecutive day. Intrassession repeatability was assessed by comparing 10 acquisitions of 3 subjects, in a row. Inter‐operator reproducibility was evaluated by comparing 10 subjects imaged by 2 operators. Inter grader reproducibiliy was evaluated by assessing 60 different images by 2 masked graders. Correlation coefficient and coefficients of variation(COVs) were calculated.Results The mean cone counts was respectively 23623±3831/mm2 and 22851±3543/mm2 for the first and the second operator (t test, p <0.05). Coefficient correlation was 0.81 between operators, and 0.97 between the 2 graders. The intrasession and intersession COVs were under 7%. The percentage of hexagonal cells and the spacing, varied in the same proportion (COV range from 1.66 to 10.05%).Conclusion Cone counts using the RTx‐1™ device and its software are reliable in normal human eyes. Further studies in the normal clinical setting are needed.