bluuebunny/crossref_metadata_2025_split huggingface dataset:
Paraneoplastic syndromes
Authors: SZATMARY G , POLGAR TATJA 
Published: November 2013

AbstractNeuro‐ophthalmologic disorders in the patient with cancer Paraneoplastic syndromes Background: Paraneoplastic syndromes are caused by remote effect of cancer rather than direct invasion. Objective: Review of paraneoplastic neuro‐ophthalmic manifestations, differential diagnosis, diagnostic workup and proposed pathophysiology will be presented. Methods: Literature review along with representative case presentations. Results: Overall incidence of remote effect of cancer is 10‐15 % of all cancers. The number of autoantibodies in association with paraneoplastic syndromes is accumulating. Conclusion: Paraneoplastic syndrome with ocular manifestation may be the first sign of malignancy, and therefore ophthalmologist may play a crucial role in early detection and timely treatment with potential improvement of the disease course.
Radiation optic neuropathy
Authors: BORRUAT F 
Published: November 2013

AbstractRadiation optic neuropathy (RON) is a radionecrosis of the anterior visual pathway. It is a rare delayed and unpredictable complication of radiotherapy. RON presents with an acute painless and permanent visual loss. Both accurate and early diagnoses are mandatory as some specific therapies might benefit the patient. This presentation will summarize the physiopathology of RON with an emphasis on clinical diagnosis and therapeutic possibilities.
High resolution retinal image analysis of unilateral retinitis pigmentosa using adptive optics
Authors: GOCHO K , KIKUCHI S , KABUTO T , AKEO K , KAMEYA S , YAMAKI K , TAKAHASHI H 
Published: November 2013

AbstractPurpose To report the findings of en face adaptive optics (AO) fundus imaging in eyes with unilateral retinitis pigmentosa(RP) patient.Methods The both eyes from a unilateral RP patient, 52 y.o. female, was imaged using both an infrared AO camera (rtx1™, Imagine Eyes, France) and spectral‐domain optical coherence tomography (SD‐OCT – Cirrus™, Carl Zeiss Meditec, Germany). Full field scotopic and phototic electroretinograms(ERGs) were recorded according to the ISCEV standard. Normal funduscopic examination, Goldmann visual field and fluorescence angiography(FA) were also examined. The AO images acquired from the unilateral RP patient were analyzed on cone density and then compared to the AO data from the eyes of 21 healthy volunteers with a mean age of 36 years (range 20‐57).Results The unilateral RP patient showed boney spicule changes, vascular attenuation and decreased peripheral visual field in the right eye. FA showed granular hyperfluorescence in the post pole, mottling of RPE and atrophy of choroid capillary in the peripheral retina in the right eye. Her ERGs of the right eye showed non‐recordable in all responses tested. OCT findings in the right eye showed preservation of the photoreceptor layer only in the foveal and parafoveal regions with loss in the more peripheral macula. All these results in her left eye were normal. AO data showed severe reduction of cone density in the right eye and the left eye showed normal cone density and regular cone mosaic.Conclusion In unilateral RP patient, AO showed only one eye was affected even in the cellular level examination. The examinations using AO may be useful in diagnosis and better understanding of pathology and management of unilateral RP cases.
Pilot study in Polish keratoconus patients using next generation sequencing
Authors: GAJECKA M , KAROLAK JA , NOWAK DM , POLAKOWSKI P , SZAFLIK JP 
Published: November 2013

AbstractPurpose Next generating sequencing (NGS) is a useful tool in disease etiology evaluation. Keratoconus is a multifactorial disease in which NGS technology may be applicable. Here we present a design of keratoconus study based on transcriptome analyzes in human corneas derived from keratoconus and control individuals from Poland.Methods A total of 96 individuals from Polish population are included into this study: 48 keratoconus patients and 48 individuals without keratoconus phenotype. Illumina HiScan SQ system is applied in transcriptome analyzes in the study.Results Total RNA from corneas for RNAseq and small RNA analyzes was extracted. Transcriptome analyzes were initiated.Conclusion Since genetic factors involved in keratoconus etiology are in majority unrecognizable, the newest available technologies are necessary to be involved in further keratoconus research. Support: Polish Ministry of Science and Higher Education, Grant no. NN402591740 and National Science Centre in Poland, Grant no. 2012/05/E/NZ5/02127.
Role of O‐GlcNAcylation of Sp1 in the pathogenesis of diabetic retinopathy
Authors: DONOVAN K , ALEKSEEV O , AZIZKHAN‐CLIFFORD J 
Published: November 2013

AbstractPurpose Central to the pathogenesis of diabetic retinopathy (DR) is vascular endothelial growth factor A (VEGF‐A). Gene expression of VEGF‐A is regulated by several transcription factors, including specificity protein 1 (Sp1). Sp1 is known to participate in angiogenesis and co‐localizes with VEGF‐A in the epiretinal membranes of DR patients. Sp1 is heavily O‐GlcNAcylated by the enzyme O‐GlcNAc transferase (OGT). This glycosylation modification affects its transcriptional activity, localization, stability, and protein interactions. Since O‐GlcNAcylation of proteins is elevated under high glucose conditions, we investigated the relationship between O‐GlcNAcylation of Sp1 and increased VEGF‐A transcription.Methods Hyperglycemia‐exposed ARPE‐19 (retinal pigment epithelial cells) and TR‐iBRB (rat retinal microendothelial cells) were assayed for levels of VEGF‐A by RT‐qPCR and Western blot. VEGF‐A protein and mRNA levels were measured in cells depleted of Sp1 or OGT by shRNA. Small molecule inhibitors of OGA and OGT were used to increase or decrease total O‐GlcNAc. Chromatin immunoprecipitation was used to assess levels of Sp1 bound to the VEGF‐A promoter.Results Hyperglycemia caused increased VEGF‐A transcript and protein expression in ARPE‐19 and TR‐iBRB cells. OGT or Sp1 depletion significantly abrogated these glucose‐induced changes in both cell types, while minimally affecting basal expression. ChIP analysis showed that glucose increased the amount of promoter‐bound Sp1.Conclusion Glucose‐induced increases in pan O‐GlcNac in the RPE and vascular retina may be participating in the aberrant expression of VEGF‐A in DR. The highly O‐glcosylated Sp1 transcription factor may be responsible for early VEGF‐A production in the retina.
Genotype‐phenotype correlation in two patients with posterior polymorphous corneal dystrophy 3
Authors: LISKOVA P , PALOS M , HARDCASTLE A , VINCENT A 
Published: November 2013

AbstractPurpose To determine the molecular genetic cause of posterior polymorphous corneal dystrophy (PPCD) in four Czech probands.Methods Extensive ophthalmological examination including Pentacam and specular microscopy, and direct sequencing of the ZEB1 coding region were performed.Results Two novel frameshift mutations within ZEB1 were identified; c.2617dup in exon 8 in a 21‐year‐old female, considered to be most likely de novo in origin, and c.698dup in exon 6 in a 21‐year‐old male. The first case had mild changes consistent with a PPCD diagnosis and best corrected visual acuity (BCVA) bilaterally was 1.0. The corneal phenotype of the second case was more severe with a BCVA of 0.4 in the right and 0.05 in the left eye. Corneas of both probands were abnormally steep (keratometry readings K1 ≥ 47.4 D and K2 45 ≥ 49.2 D) with increased pachymetry values, but no pattern indicative of keratoconus. Specular microscopy in both patients revealed reduced endothelial cell density (range 1055‐ 655 per mm2). Both probands had a history of surgery for inguinal hernia; the male patient also reported the presence of hydrocele.Conclusion Nucleotide changes within the coding region of ZEB1 underlie the pathogenesis of PPCD in 4 out of 23 (17.4%) Czech probands.
The role of wound healing in cornea
Authors: BEUERMAN R 
Published: November 2013

AbstractCorneal fibrosis contributes to the substantial health burden due to “corneal blindness”, a leading cause of blindness world‐wide. Molecular intra‐cellular regulators of the cytoskeleton, suggests that this class of proteins may be targets for therapeutic development. In the transformation the usually quiet fibroblast‐to‐a myofibroblast morphology, the expression of α‐smooth muscle actin (SMA) is characteristic. As an excessive healing response, fibrosis is the result of the corneal keratocyte to myofibroblast transformation and may occur in response to trauma, infection as well as surgery. Using a mouse model of fibrosis following both infection with Pseudomonas and by trauma (an anterior keratectomy) it was shown using an array for the cytoskeletal regulators that one key molecule was highly unregulated. Moesin a member of the ERM Complex (ezrin/radixin/moesin) was highly unregulated. The use of siRNA applied by iontophoresis showed that the molecules characterizing fibrosis, such as alpha‐SMA. The clinical development focuses on a method that can be applied topically to lessen or prevent fibrosis after corneal insult.
The mouse model of glaucoma filtration surgery: from understanding the wound healing response to applications as a tool for validating anti‐fibrotics
Authors: LI‐FONG LF , WONG TT 
Published: November 2013

AbstractPurpose: Failure after glaucoma filtration surgery (GFS) is attributed to fibrosis at the operated site. We have developed a mouse model for GFS so as to understand the wound healing process after this procedure and to test anti‐fibrotics for future clinical application. Methods: Modified GFS was performed on mice and bleb survival was determined by slit lamp photography. Real‐time PCR, antibody‐based cytokine assays, flow cytometry, and immunofluorescent analyses were performed to determine gene and cellular changes. Results: The mouse model of GFS mimicked the clinical response to mitomycin C. Further analysis of the operated tissues revealed that the post‐surgical wound healing response was separable into two distinguishable phases: an early “acute inflammatory” phase followed by a late “fibrotic” phase. Conclusions: Our data suggest that targeting the distinct phases of the wound healing process after GFS may be desirable in order to overcome the long term scarring response. Furthermore, the mechanisms of potential anti‐fibrotics may now be understood in terms of their specific effects on each phase of the wound healing process. Paper presentation at Anatomy & Cell Biology, Special Interest Symposium.
Choroidal thickness measurements and variations with age, gender in normal, myopia and high myopia
Authors: COSCAS G 
Published: November 2013

AbstractTo evaluate CT and variations along foveal horizontal scan and age related change in CT in normal and myopic Methods:Multi‐center prospective study with 3 groups:30 Normal,24 Mild Myopia,31 High Myopia. Manual measurement followed choroid boundary selection(supra‐choroid space,lamina fusca and outer limit of vessels. CT was measured at 500 µm intervals up to 1,500 µm temporal and nasal, manually by two graders independently. Statistical analysis evaluated CT at each location,the effects of age and myopia and grader agreement.Results: 85 eyes were qualified.Excellent grader agreement:intra‐class correlation>0.97(Bland‐Altman analysis).The mean CT was 248.2±78.5µm for Normal‐‐age=58±18;247.0±85.4 for Myopia‐age=45±20;131.5±70.9for High Myopia‐age=54±13. The mean CT was significantly thicker for Normal and Myopia than High Myopia. CT was highly correlated across all test locations and slightly thicker underneath the fovea. The overall slope of age related mean change for the mean CT was ‐1.95 µm/year.The effect of age differed among the study groups.CONCLUSION: OCT measurements of CT are reproducible.Age and high myopia negatively affect CT.There was good agreement in CT measurement between RTVue and Spectralis.
Choroidal circulation measurements by laser Doppler flux metry
Authors: POURNARAS CJ 
Published: November 2013

AbstractContinuous real time Laser Doppler Flowmetry (LDF), has been used in humans, for the evaluation of the subfoveal choroidal circulation and the investigation of the regulatory processes of the choroidal blood flow in response to various physiological stimuli, as increases and decreases of the ocular perfusion pressure. A progressive decrease of the choroidal blood flow was observed with the aging and the progession of the age related macular degeneration. Abnormal regulation of the choroidal blood flow in patients suffering with neovascular AMD or Diabétic microangiopathy were also reported. The correlation of the subfoveal choroidal blood flow with the anatomical changes of the choroidal circulation as observed by OCT are under investigation.
Evaluation of posterior limits of choroid
Authors: UZZAN J 
Published: November 2013

AbstractPurpose: Recent studies have reported choroidal thickness measurements based on FD‐OCT images. Lamina fusca (LF) is the natural anatomical outer boundary of the choroid. Unfortunately, LF is not always clearly visible in OCT images. The goal of this study is to assess the difference between LF boundary and posterior choroidal vessel (PCV) boundary. Methods: In a retrospective case review study, Horizontal B‐Scan images, through the center of the fovea were reviewed and 96 eyes were included. The eyes were divided into 3 categories based on the characteristics of the choroid‐sclera junction: SCS if suprachoroidal space is visible as a dark strand, LF if lamina fusca is visible as a bright strand, and PVC if only posterior vessel cavity boundary is visible. Results: For the 48 eyes in either SCS or LF, choroid thickness measurement differ by 26.9μm on average, ranging from 8 to 85 μm in individuals between the two boundaries. Conclusions: Choroid thickness measurement could be affected by boundary criteria. Posterior vessels boundary is the best method in 50% of cases but the presence of Supra Choroidal Space must be remembered. A common posterior boundary definition may be necessary to avoid significant bias in further studies.
Comparison of choroidal thickness between central serous choroidopathy and polypoidal choroidal vasculopathy
Authors: PUCHE N , COSCAS F 
Published: November 2013

AbstractPURPOSE: To compare choroidal thicknesses among eyes with polypoidal choroidal vasculopathy (PCV), and central serous chorioretinopathy (CSC). METHODS: Patients with PCV and CSC underwent enhanced depth imaging optical coherence tomography (EDI OCT). A horizontal linear section comprising 100 averaged scans was obtained of each macula. The choroidal thickness was measured from the outer border of the retinal pigment epithelium to the inner scleral border. Seventeen healthy subjects (17 eyes) also underwent EDI OCT. RESULTS: A total of 17 eyes of 17 consecutive patients with CSC (17 male, mean age 50 years range 37‐66 years) and 15 eyes of 15 patients with PCV were included in the analysis (8 male, mean age 69 years range 57‐73 years). Mean choroidal thickness was 437 µm ± 97 µm in patients with CSC and mean choroidal thickness was 335 µm ± 53 µm in patients with PCV. The choroid was thicker in PCV and CSC patients than in controls(mean age 62 years range 55‐72, 219 ± 35 µm p<0.005). However, there was significant difference in mean subfoveal choroidal thickness between CSC and PCV (p<0.005) . CONCLUSION: The choroid was thicker in eyes with PCV or CSC than in control
Clinically sensible follow‐up for glaucoma
Authors: LEMIJ H 
Published: November 2013

AbstractDetecting glaucoma progression is clinically challenging. It is unclear how structural and functional measurements are best used. Data from several long‐term structure and function follow‐up studies into glaucoma progression have revealed a poor overlap between the two, suggesting that one should ideally use both structural and functional measurements for detecting glaucomatous progression in individual cases, with the risk of overcalling and overtreating so‐called progression. With ever more limited resources, however, clinicians are probably forced to restrict their measurements to as few exams as possible in individual patients, calling for sensible choices which measurements to perform. Structural measurements appear to be more useful in early, including preperimetric, glaucoma and functional measurements in more advanced glaucoma. Descriptions, drawings or 2D photography of the optic disc are, on the whole, probably of limited value, whereas standard automated perimetry and imaging of the retinal nerve fibre layer appear to be most useful. This will be discussed in an interactive, clinically oriented presentation. A limitation of this study is that scanning laser tomography of the optic disc has not been evaluated.
Optical measurement of dioptric power and transparency of cornea stored in bioreactor
Authors: PATAIA G , GAUTHIER AS , TRONE MC , CAMPOLMI N , BERNARD A , NAIGEON N , NANGOUM‐FOSSO T , PISELLI S , ACQUART S , LEPINE T , DELBOSC B , THURET G , GAIN P 
Published: November 2013

AbstractPurpose Quantification of dioptric power (DP) and transparency (T) of stored corneas remain almost unmet in daily eye bank practice. Complementary to T, DP could help screening donors having had refractive surgery and selecting the best donor/recipient refractive matching. During the development of an innovative corneal bioreactor (BR) (patented) for preclinical research and long‐term storage, we took into account the possibility to integrate devices to perform these new quality controls. Aim: to present a device, named “frontofoco‐graft‐meter” (FGM) able to quantify DP and T of corneas in BRMethods measure of DP was based on the principle of magnification measurement and for T on the transmitted light over incoming light ratio. The FGM comprised a light source + specific chart made of parallel black lines + digital camera. Human corneas in the BR were pressurised at 15mmHg, with medium circulation in endothelial chamber, whereas epithelial chamber was filled with air during measurements. DP was compared to the power map provided by OCT (Tomey, Casia), and T measures to results provided by our previous device called ‘transparometer’ (based on the analysis of modulation transfer function). Measurements were done by 3 operators in a masked fashionResults For DP, agreement with OCT was good (within ±1 Dioptry). For T, measures were comparable with both devices. Inter‐rater agreement was good for DP and T. FGM took a limited place and could easily be integrate in eye banksConclusion the new FGM provides objective and reproducible QC of DP and T of corneas placed in the BR. Grants: ABM 2012, ANSM 2012, EFS 2012
Endothelial and stromal quality control of corneas stored in an innovative bioreactor
Authors: GAUTHIER A , TRONE MC , CAMPOLMI N , NEFZAOUI C , BERNARD A , NAIGEON N , NANGOUM‐FOSSO T , PISELLI S , DELBOSC B , ACQUART S , THURET G , GAIN P 
Published: November 2013

AbstractPurpose We recently patented a bioreactor (BR) for corneal storage, based on the restoration of a transcorneal pressure gradient and a continuous fluid circulation. Being designed for experimental works and eye banking it comprises 2 windows enabling a right through observation of the cornea. Aim: to assess on human corneas placed in the BR, the possibilities of non‐invasive quality controls (QC) of endothelial cells (EC) and of the stroma (thickness, dioptric power, transparency, detection of scars)Methods EC QC were: 1/transmitted light microscopy (TLM) with a x10 very LWD objective after trypan blue + osmotic preparation directly performed in the BR; 2/observation with a macroscope (same preparation); 3/specular microscopy (SM, Konan and Hai) without preparation. Stromal QC were: 1/slit lamp; 2/OCT (Heidelberg and Tomey Casia); 3/customized transparometer (analysis of modulation transfer function), and 4/macroscope. Both quality of results and ergonomic aspects were consideredResults EC were visible with TLM and macroscope at highest magnification but not with SM. Casia OCT allowed easy thickness mapping with a good agreement with US pachymetry performed as a control, and dioptric power measurements. Transparency could be controlled with the slit lamp and with our transparometer. The macroscope visualized stromal scarsConclusion EC and new stromal QC are now possible for corneas stored in the innovative BR. Other new complementary technologies of optical control are currently in development in our lab. Acknowledgments: EFS of Besançon Grant: ABM 2012, EFS 2012, ANSM 2012. PG, GT and SA patented the BR
Congenital hypertrophy of the retinal pigment epithelium (RPE)
Authors: ZOGRAFOS L 
Published: November 2013

AbstractCongenital hypertrophy of the Retinal Pigment Epithelium (CHRPE) is a tumour which may be confused with pigmented tumours of the choroid such as nevi or melanomas. These lesions are round or oval shaped, with borders that are well defined or scalloped. They present areas of atrophy which may increase in size. This increase in size of CHRPE is well documented and innocent. However, transformation in adenomas or even adenocarcinomas is also possible. In consequence, a periodical observation for Congenital hypertrophy of the Retinal Pigment Epithelium is generally recommended.
Controversies in the surgical treatment of combined hamartomas of the retina and retinal pigment epithelium
Authors: GARCIA‐ARUMI J , VELAZQUEZ VILLORIA D 
Published: November 2013

AbstractCombined hamartomas of the retina and retinal pigment epithelium are rare benign tumors that may cause significant visual loss. Combined hamartomas are usually solitary, unilateral lesions located at the optic disc or posterior pole. They typically appear slightly elevated and have varying amounts of pigmentation, vascular tortuosity and epiretinal membrane formation. They are usually diagnosed in children or young adults with painless visual loss. Visual function varies with the location of the lesion. Direct involvement of the optic nerve, papillomacular bundle, or fovea may reduce visual acuity. If these structures are not directly involved, visual loss may result from macular distortion by retinal striae and epiretinal membrane. According to the Macula Society’s1 report of 60 patients with combined hamartomas, the tumor was located on the optic disc and adjacent retina in 18% of patients, adjacent to the disc in 28% of patients, extending from the disc to the fovea in 10% of patients, in the macula in 38% and in the mid‐periphery in 5% of patients. Macular location of the tumor is a predictor of poor visual acuity, found in 69% of macular tumors and only 25% of extramacular tumors. Histopathologically, combined hamartomas show evidence of hamartomatous malformation involving hyperplasia of RPE, glial cells, and blood vessels. Epiretinal membranes are an important cause of progressive visual loss, especially in eyes with macular tumors. Indication for epiretinal membrane removal in these patients is not well stablished, and cases reported in literature show different visual results. McDonald et al2 indicated that vitrectomy was not beneficial in their 2 cases, whereas Mason3, Kleiner3 and Stallman4 found benefit in their cases. The surface glial membrane causing the retinal folding is an integral part of the tumor in some cases and accounts for the fact that surgical stripping of the membrane is difficult and has little chance of restoring central vision in such cases5. Surgical intervention should be selected carefully; full‐thickness macular holes have occurred when the membrane is intrinsically woven into the lesion6. It has been speculated that OCT could provide useful information regarding potential visual benefit as the more intact retina on OCT might have better visual outcome than the completely disorganized, thickened retina7. Vitreous traction if present may also be detected, and thus help in selecting cases that may have a potential for visual improvement with vitrectomy and membrane peel to relieve traction.