bluuebunny/crossref_metadata_2025_split huggingface dataset:
The particularities of corneal infectious diseases in children
Authors: BREMOND‐GIGNAC D 
Published: November 2013

AbstractOcular surface bacterial infections affect subjects of all ages with a high frequency in newborns and children. In infant, children and teenagers, the most common ocular pathogens, which differ from adults, are Haemophilus influenzae, Staphylococcus aureus, Streptococcus pneumoniae and also Moraxella species. These infections could lead to ulcers and sight‐threatening complications. Corneal infections are not common but can be difficult to diagnose because the pathology can develop without pain. In addition the examination may be difficult to perform. The treatment should be provided earlier and aims to eliminate the bacteria, virus or fungal pathogens. The specific epidemiology of pathogens will be detailed according to the age. Risks of visual impairment and amblyopia must be integrated. An update on topical antibiotics and current options will be reviewed with practical aspects, diverse clinical cases and considering quality of life of the children and parents.
iStent implant
Authors: AU L 
Published: November 2013

Abstractistent is a novel device that canulates Schlemms canal to enhance physiological outflow and lowers intraocular pressure. Results of istent surgery combined with phaco would be presented, together with the results of intraoperative flow test. Results of multiple istents and istent Supra would also be presented. New device like the Hydrus would also be demonstrated.
Commercial interest
Neuropeptide Y Y1 receptor is neuroprotective and modulates microglia reactivity in the rat retina
Authors: AMBROSIO AF , ELVAS F , MADEIRA M , BRUDZEWSKY D , MARTINS J , MARTINS T , CAVADAS C , SANTIAGO AR 
Published: November 2013

AbstractPurpose Neuropeptide Y (NPY) is neuroprotective and can regulate inflammation. In the retina, microglia (MG) activation has been implicated in the pathogenesis of several diseases. We investigated whether NPY, particularly through Y1 receptor (Y1r) activation, is neuroprotective and modulates pro‐inflammatory responses in the retina.Methods Immunocytochemistry, RT‐PCR, TUNEL and ELISA were used.Results NPY and NPY Y1, Y2 and Y5 receptors were detected in retinal ganglion cells (RGC) and MG. The increase in TUNEL‐positive cells in the ganglion cell layer induced by exposure of retinal explants to NMDA was inhibited by Y1r activation. Exposure to LPS activated retinal MG, and NPY prevented MG morphological changes via Y1r. NPY or [Leu31, Pro34]‐NPY (LP‐NPY; Y1r/Y5r agonist) inhibited the increase in iNOS immunoreactivity triggered by LPS, and BIBP 3226 (Y1r antagonist) abolished the effect of LP‐NPY. LPS also increased the concentration of TNF‐alpha, IL‐1beta and IL‐6. NPY inhibited the increase in IL‐1beta and IL‐6, but not TNF‐alpha. Moreover, LP‐NPY inhibited the increase in TNF‐alpha, IL‐1beta and IL‐6, and BIBP 3226 abrogated the effect of LP‐NPY.Conclusion In conclusion, RGC and MG express NPY and NPY Y1, Y2 and Y5 receptors. The activation of Y1r can exert neuroprotective effects in RGC and induce anti‐inflammatory effects in the retina, inhibiting MG activation. Support: FCT (PTDC/SAU‐NEU/099075/2008, PTDC/NEU‐OSD/1113/2012, PEst‐C/SAU/UI3282/2011‐2012, PEst‐C/SAU/LA0001/2011‐2012, SFRH/BPD/69123/2010, SFRH/BD/44817/2008), Portugal, and COMPETE‐FEDER.
Rescue therapy with subcutaneous anti‐TNF‐α agents for idiopathic non‐infectious uveitis
Authors: NERI P , ARAPI I , PIRANI V , CAPUANO V , GIOVANNINI A , MARIOTTI C 
Published: November 2013

AbstractChronic non‐infectious uveitis (NU) remains one of the most challenging problems in ophthalmology. Often, early and aggressive treatment is needed for a good visual acuity outcome. Local and oral corticosteroids remain the first line of treatment, even though side effects, such as glaucoma, cataract and Cushing Syndrome, can be serious and not tolerable. Therefore, a variety of immunosuppressive agents are used. Recently, new types of drugs, called “biologic agents”, are available. In the model of experimental autoimmuneuveitis (EAU) it has been demonstrated that tumor necrosis factor‐alpha (TNF‐α) may play a key role in uveitis. Beside the well known intravenous anti‐ TNF‐α agent called infliximab, other sub cutaneous anti‐ TNF‐α drugs have been introduced, such as Adalimumab and Golimumab. Adalimumab presents a stronger medical literature and it seems to present promising qualities. Few reports are available for Golimumab in uveitis at this time. Adalimumab can control uveitis unresponsive to the traditional immunosuppressive agents and, moreover, its subcutaneous administration makes the procedure user friendly. This drug can be used in selected cases as a rescue therapy for idiopathic non‐infectious uveitis.
A general introduction to the tests and abnormality localisation
Authors: LEROY B 
Published: November 2013

AbstractPurpose: To illustrate which electrophysiological tests are available to the visual electrophysiologist, and to highlight when and where they can be used to learn more about the patient's condition. Methods: A systematic overview of the different electrophysiological tests will be given, including a physiological and anatomical correlation for each test. Results: Each of the different electrophysiological tests available allows the clinician to evaluate the function of different aspects of the retina and/or optic nerve. In so doing, essential information is obtained in order to pinpoint the exact location of the dysfunction, and make a correct diagnosis. Conclusions: Visual electrophysiology is essential to make a correct diagnosis both for acquired and inherited retinal and optic nerve diseases.
Electrophysiology in inherited disease
Authors: LEROY B 
Published: November 2013

AbstractPurpose: To illustrate the added value of visual electrophysiology in the diagnosis of inherited retinal dystrophies and dysfunctions, and their management in the ophthalmic genetic clinic. Methods: A systematic overview with a case presentation format will be used to illustrate different genetically determined retinal dystrophies and dysfunctions. Case descriptions will include clinical and electrophysiological phenotypes as well as genotypes. Results: Phenotypes and genotypes of genetically determined retinal diseases are very different, and visual electrophysiology is essential in making a correct diagnosis. Conclusions: Taking a thorough history in combination with an extensive clinical examination and psychophysical and electrophysiological tests most often allows a to make a specific diagnosis when dealing with inherited retinal disease. Visual electrophysiology also allows to distinguish between progressive and stationary conditions, and it provides essential information regarding evolution of progressive disease.
Gain of chromosome 6 status does not influence HLA‐expression in uveal melanoma
Authors: VAN PELT S , VAN ESSEN TH , BRONKHORST IHG , VERSLUIS M , LUYTEN GPM , VAN HALL T , VAN DEN ELSEN PJ , VAN VELDEN PA , JAGER MJ 
Published: November 2013

AbstractPurpose Chromosomal aberrations and the inflammatory phenotype have been identified as predictive factors for survival of uveal melanoma (UM). The mechanism by which these factors are linked remains obscure. We took the human leukocyte antigen (HLA), located on chromosome 6p as marker for inflammation and studied whether aberrations of chromosome 6p influenced the HLA expression in UM.Methods SNP‐array copy number analysis and gene expression profiling were performed on 28 uveal melanomas of patients who underwent enucleation between 1999 and 2004 at the Leiden University Medical Center, Leiden, in The Netherlands. UM protein expression of HLA‐A, ‐B and –DR was measured with immunohistochemistry. The status of chromosome 3, 6p, and gene expression of HLA and HLA regulators were analyzed, as well as protein expression of HLA.Results Gain of 6p was present in 8 cases (29%) and not related with survival. An increased gene expression was seen in the group who died due to metastatic UM for HLA‐A and –B, and B2M. Gene expression correlated with protein expression for HLA‐A, HLA‐B, but not with HLA‐DR. Tumors did not differ in level of HLA protein expression, HLA gene expression, and HLA regulator gene expression with regard to chromosome 6p status.Conclusion High HLA gene‐ and protein expression in UM are not influenced by gain of 6p. Yet for the first time we demonstrated that in UM an increased expression of HLA class I genes correlated with the elevated protein expression of HLA class I.
Ophthalmic histopathology samples – are we sending enough?
Authors: DEV BORMAN A , HASSANI M , KESHARAJU R , NADERI K , GHAZI‐NOURI S 
Published: November 2013

AbstractPurpose To ascertain the range of histopathological diagnoses made from tissues removed during ophthalmic surgical procedures over two years from one NHS Trust. During this period, joint recommendations were published by the Royal College of Pathologists (RCPath) and the Royal College of Ophthalmologists (RCOphth) in the United Kingdom, regarding the referral of ophthalmic pathology specimens, stating that in order to avoid delayed or missed diagnoses, with certain exceptions, all tissues obtained from ophthalmic surgical procedures should be sent for histopathological examination. This policy was implemented locally from January 2011.Methods This was a retrospective case note review of the ophthalmic histopathology specimens sent between January 2010 and December 2011, at the Mid Essex Hospital Services NHS Trust. Patient demographics and histopathological diagnoses were determined for each.Results Over the two‐year period, 268 specimens were sent for ophthalmic histopathological examination. In 2010, 112 samples were sent, with 18 malignancies detected (16.1%). In 2011, 156 samples were sent, with 30 malignancies identified (19.2%). The most common diagnosis was basal cell carcinoma, comprising 43/48 malignancies (89.6%).Conclusion Since the local implementation of the joint guidelines from the RCPath and RCOphth, there was an increase in the number of ophthalmic histopathology specimens sent within this Trust, with a 3.1% increase in the number of malignancies detected. We believe that this improvement in detection of ocular malignancies justifies the increased burden on the histopathology service from the ophthalmology unit within this Trust.
Orbital melanocytosis and OTA naevus
Authors: DE KEIZER RJW , LAUWERS N , DE GROOT V 
Published: November 2013

AbstractPurpose Presentation and discussion of a rarely reported location of orbital melanocytosisMethods Case reportResults In a 54 year old man with an oculodermal melanocytosis (naevus of OTA), enucleation was performed for a choroidal melanoma. During the surgery spots of hyperpigmention, based on melanocytosis, were found in tendons, muscles and orbital fat. Oculodermal melanocytosis is classically described on the periocular skin, sclera, uvea, orbit, meninges, palate or tympanic membrane and is a well‐known risk factor to develop choroidal and orbital melanoma. Few reports describe the particular involvement of the orbital tissue. We show the pictures of orbital tissue and extraocular muscle pigmentation and explain it with embryology.Conclusion Even after enucleation for a choroidal melanoma in an OTA naevus, the ophthaist should be alert that extraocular melanocytes can remain and potentially be the cause of an orbital melanoma.
Functional assessment of macular edema
Authors: MIDENA E 
Published: November 2013

AbstractAim Macular edema is evaluated using pure morphologic imaging tests and visual acuity as sole visual function test. Unfortutanetly, visual acuity just partly reflects visual function. A new integrated morpho‐functional approach seems useful. Methods Patients affected by macular edema were investigated using a multimodal/integrated imaging approach including currently available pure morphological retinal imaging techniques and microperimetry. Results The correlation between morphologic and functional imaging modalities strictly depends on the different involvement of retinal layers by the pathologic process. Data shows that visual function impairment is better quantified when microperimetry is added to the currently available diagnostic armamentarium. Conclusions Both in clinical trials and in daily clinical practice visual function needs to be better investigated to cope with patient’s requests. Adding reliable, topographically related visual functional data, currently obtained by microperimetry, to other pure retinal imaging investigations allows not only to better identify different “clinical phenotypes “ of macular disorders, but also to provide invaluable information about treatment and retreatment criteria.
How to select our treatments?
Authors: CREUZOT C 
Published: November 2013

AbstractThe analysis of associated circumstances to macular edema will help the clinician to select one among the different therapeutic options of macular edema (ME). The duration of ME, the efficacy of previous treatments, the ability of the patient to be examined regularly will influence this choice. The different options are grid laser, antiVEGF agents, steroids. Associated risk factors like severe glaucoma or lens status also need to be considered. The control of systemic conditions remain a key issue. The strategy can change during the follow‐up according to the efficacy and the recurrences observed with the treatments.
Commercial interest
Differnetial diagnosis
Authors: ANSARI E 
Published: November 2013

AbstractThere are many conditions that can mimic glaucoma. It is important to be aware of the differential diagnosis so that other conditions that can present with visual field defects and/or optic neuropathy can be detected and dealt with promptly. External ocular, retinal, optic nerve, systemic and visual pathway problems can present with features of glaucoma. A careful history and thorough examination will guide the clinician to making the correct diagnosis. Particuar emphasis should be made on the pattern of visual field defects, the optic disc cupping versus palor and whether the disc appearance correlates with the visual field defect. Timely medical advice should be sought to avoid sight/life‐threatening conditions in some of these cases.
Counselling in advanced glaucoma
Authors: ANSARI E 
Published: November 2013

AbstractThe loss of useful vision can be a very emotional experience for the patient and a massive challenge for the clinician. More time and a team‐orientated approach is vital in order to support the patient through such a crisis. Disciplines such as counselling are an essential part of the mamagement process for a patient with impaired vision and they can give advice on matters such as daily living, benefits, support groups and safety around the house. We have learnt that a separate multi‐disciplinary consultation away from the hustle and bustle of the main clinic is very helpful for the patient, relatives and carers.
Rationale for a diagnostic approach
Authors: GAVARD‐PERRET A , ROBERT PY 
Published: November 2013

AbstractPurpose: to propose a diagnostic approach for clinicians who manage a patient with non Graves’ orbital inflammation. Patients&Methods: a review of the diagnostic process for 61 patients with diagnostic of orbital inflammation between january 2002 and january 2013 at Nice and Limoges Universitary Hospitals. Results: sixty one patients presenting with orbital inflammation were included. More common presentations were orbital fat inflammation (60%), myositis (26%) and lacrimal gland enlargement (18%). In few cases, optic nerve and sclera were involved (respectively 9,8 and 3,2%). The final diagnostic was idiopathic orbital inflammation (25 patients), specific orbital inflammation (17 patients) and orbital lymphoma (19 patients). Thirty six patients had biopsy first and 25 patients did not, in first, but only after failure to the initial therapy. Our results showed that doing biopsy in the initial management of orbital inflammation provides a more great number of specific diagnosis than doing treatment tests directly after blood tests or CT scans( p>0,01). Conclusions: The diagnostic process of orbital inflammation must include blood testing, imaging of the orbit and sinuses, and biopsy in first intention as far as possible.
ICGA features in VKH
Authors: BOUCHENAKI N , HERBORT CP 
Published: November 2013

AbstractIndocyanine green angiography (ICGA) is a highly sensitive method to evaluate choroidal inflammatory lesions. We present standardized ICGA findings in Vogt‐Koyanagi‐Harada (VKH) disease. Six ICGA signs including choroidal perfusion inhomogeneity, early hyperfluorescent stromal vessels, hypofluorescent dark dots (HDDs), fuzzy or lost pattern of large stromal vessels, disc hyperfluorescence, and diffuse late choroidal hyperfluorescence identified earlier were analyzed. The most constant findings present in the acute phase were four: early hyperfluorescent stromal vessels, HDDs, fuzzy/ lost pattern of large stromal vessels and disc hyperfluorescence. These angiographic signs represent a precise and universal tool to precisely assess choroiditis and follow‐up VKH patients in the acute phase as well as in subacute disease, knowing that treatment of occult choroiditis in VKH is the key to avoid sunset glow fundus and even to “heal” VKH cases when treatment is given early and vigorously during a prolonged period.
The outcomes of mycophenolate mofetil therapy combined with systemic corticosteroids in acute uveitis associated with Vogt‐Koyanagi‐Harada disease
Authors: ABU EL ASRAR A 
Published: November 2013

AbstractPurpose: To study the effectiveness of mycophenolate mofetil (MMF) as first‐line therapy in acute uveitis associated with Vogt‐Koyanagi‐Harada (VKH) disease. The outcomes in this group were compared with those of another group of patients who were treated with corticosteroid monotherapy. Methods: 19 patients(38 eyes) diagnosed with acute uveitis associated with VKH were included prospectively. Results: The mean follow‐up period was 27.0±11.1 months. Corticosteroid‐sparing effect was achieved in all patients. Ten patients discontinued treatment without relapse of inflammation. Visual acuity of 20/20 was achieved by 37.5% of the eyes in the corticosteroid group and by 73.7% in the corticosteroid + MMF group. Recurrent inflammation of 3 times or more was reduced significantly in the corticosteroid + MMF group as compared to corticosteroid group. Development of all complications was significantly higher in the corticosteroid group (42.6%) compared to the corticosteroid + MMF group (7.9%). None of the eyes in the corticosteroid + MMF group developed “sunset glow fundus”. Conclusions: Addition of MMF as first‐line therapy to corticosteroids improves clinical outcomes in patients with acute uveitis associated with VKH disease.
The expanding clinical spectrum of dominant optic atrophy
Authors: YU‐WAI‐MAN P 
Published: November 2013

AbstractAutosomal dominant optic atrophy (DOA) is the most common inherited optic nerve disorder in the population and OPA1 is the major causative gene, accounting for about 60% of families. Visual loss starts in early childhood secondary to the highly tissue‐specific loss of one cell type – the retinal ganglion cell. Although progressive visual failure remains the defining feature of DOA, we recently described the expanding phenotypic spectrum associated with OPA1 disease. Up to 20% of mutational carriers developed a more severe “DOA+” phenotype characterised by prominent neuromuscular features such as deafness, myopathy, peripheral neuropathy, ataxia, and chronic progressive external ophthalmoplegia. Interestingly, we found a three‐fold increased risk of developing the more severe DOA+ phenotype with missense OPA1 mutations involving the GTPase domain compared with other mutational subgroups. Histochemical and molecular characterisation of skeletal muscle biopsies revealed the presence of cytochrome c oxidase deficient fibres and clear evidence of mitochondrial DNA instability. Dissecting the disease mechanisms leading to optic atrophy and multisystem tissue involvement in DOA will have important therapeutic implications.
Sensorineural hearing loss in OPA1‐linked disorders
Authors: BONNEAU D , MILEA D , LERUEZ S , DEFOORT‐DHELLEMMES S , CROCHET M , LAMBLIN J , LENAERS G , PROCACCIO V , REYNIER P , BONNEAU‐AMATI P 
Published: November 2013

AbstractAbout 20% of OPA1 mutation carriers have complicated forms of dominant optic atrophy associating extra‐ocular features. Reviewing the files of 327 patients with an OPA1 mutation, we found 21 deaf patients (6.4%). In 10 patients deafness was detected under age 20, in 3 patients over age 20 and in 8 patients the age of onset was unknown. The severity of deafness ranged from mild and moderate. Audiological tests supported the diagnosis of auditory neuropathy in 8 cases. Seven different OPA1 mutations were identified in deaf patients. Three mutations, p.Arg445His, p.Gly401Asp and p.Leu243*, have been previously reported in patients with deafness. Two mutations, p.Val291_Phe328del and p.Ile463_Phe464dup, have been reported in patients with DOA without deafness. Finally, two novel OPA1 mutations, p.Arg437Glu and p.Ala357Leufs*4 were found. In the majority of patients with DOA and deafness, visual impairment occurred during the first decade while deafness appeared later. However, in 54% of patients deafness started prior to visual abnormalities. These observations suggest that audition should be carefully tested, including a specific search for auditory neuropahy in OPA1 mutation carriers.
Crosstalk of proteasomes and autophagy in RPE cells
Authors: KAARNIRANTA K 
Published: November 2013

AbstractThe pathogenesis of AMD involves impaired protein degradation in RPE cells. The ubiquitin‐proteasome pathway and the lysosomal pathway including autophagy are the major proteolytic systems in eukaryotic cells. Prior to proteolysis, heat shock proteins (HSPs) attempt to refold stress‐induced misfolded proteins and thus prevent the accumulation of cytoplasmic protein aggregates. Recently, p62/sequestosome 1 has been shown to be a key player linking the proteasomal and lysosomal clearance systems.The aging characteristics of the RPE involve lysosomal accumulation of lipofuscin and extracellular protein aggregates drusens. Molecular mechanisms behind protein aggregations are weakly understood. There is intriguing evidence suggesting that p62, together with autophagy, seems to have a role in the pathology of different degenerative diseases. In ARPE‐19 cells proteasomal inhibition caused accumulation of p62 bound irreversibly to perinuclear protein aggregates. The addition of the AMPK activator AICAR was pro‐survival and promoted cleansing by autophagy of the former complex. Human AMD donor samples showed strong p62 accumulation in the drusen rich macula revealing impaired autophagy flux.