bluuebunny/crossref_metadata_2025_split huggingface dataset:
A case of bilateral idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) treated with discontinuous immunosuppressive therapy
Authors: GONZALES S , RAMPINI A , RUBINO P , MORA P , ORSONI JG 
Published: November 2010

Abstract Purpose To describe a case of bilateral Idiopathic Retinitis,Vasculitis,Aneurysms and Neuroretinitis(IRVAN)successfully treated with systemic immunomodulatory treatment including steroid, cyclosporine and methotrexate. Methods A 47‐year‐old Italian woman was diagnosed with IRVAN at our Service.She underwent extensive general and laboratory evaluation.Periodical ophthalmologic evaluations included best correct visual acuity(BCVA)assessment,slit lamp and fundus examination.Ocular alterations were documented by fluorescein and green indocyanine angiogram,and optical coherence tomography. Results At her first examination BCVA was 20/32 in the right eye(RE)and 20/40 in the left eye(LE).In both eyes angiography showed retinal vasculitis with arterial aneurysms and moderate ischemia,mild papillitis and macular oedema.The patient started a systemic steroid spearing immunomodulatory treatment.After 12 months of follow‐up BCVA was 20/25 in the RE and 20/20 in the LE.Imaging showed a complete resolution of the retinal and optic nerve head active inflammation in both eyes,with significant reduction of macular oedema. Conclusion IRVAN is an isolated retinal vascular disease that can progress to severe visual loss due to ischemic sequelae.Ocular inflammation is likely related to immunitary disregulation,even if the aetiology is still not clear.In the presented case the bilateral involvement with papillitis and the progression of the visual loss in spite of previous local and systemic steroid regimen, were the reasons that convinced us to propose a systemic immunomodulatory treatment.This treatment was well tolerated, with a complete resolution after one year of follow‐up.
Optical coherence topographic features in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN)
Authors: NAITHANI P , VASHISHT N , SANKARAN P , MANDAL S 
Published: November 2010

Abstract Purpose To evaluate Optical Coherence Tomography (OCT) findings in Idiopathic retinitis, vasculitis,aneurysms and neuroretinitis (IRVAN). Methods In this case‐series, 3 patients with IRVAN were evaluated. The patients underwent complete ophthalmological examination including fundus photography, fluorescein angiography, and OCT. Main outcome measures: central macular thickness(CMT) & associated morphological changes in different retinal layers on OCT. Results OCT scans of all patients demonstrated diffuse retinal thickening with loss of normal foveal contour. The increased thickness in 2 patients was associated with focal areas of hyper‐reflectivity with high backscattering of inner layers in macular area suggestive of macular exudation. In third patient, OCT images demonstrated vitreomacular traction(VMT) along with exudative maculopathy. Epiretinal membrane(ERM) was noted in 2 patients. Conclusion OCT is a valuable tool in evaluating macular changes in IRVAN. It is helpful in detecting early subclinical findings like VMT and ERM & provides further insight into causes of visual loss in IRVAN.
Xanthophylls from blood to retina
Authors: BRETILLON L 
Published: November 2010

AbstractXanthophylls are dietary lipophilic compounds. Among them, lutein and zeaxanthin are the major carotenoids found in the human lens and retina, and referred as macular pigment within the retina. Lutein and zeaxanthin cannot be synthesized endogenously. They may therefore be considered as essential and must be provided by adequate dietary intakes. Lutein and zeaxanthin are present in various food items, mainly in plants and fruits such as green vegetables or yellow‐orange fruits, as well as in a few animal sources, such as egg yolk. Epidemiological studies consistently suggest that dietary lutein and zeaxanthin are protective factors against the development of Age‐Related Maculopathies and Age‐related Macular Degeneration. Intervention trials consisting in supplementing the diet with lutein and zeaxanthin demonstrate the bioavailability of those carotenoids in plasma and, in some of them, their efficacy in increasing the density of the macular pigment. An overview will be presented on the mechanisms of xanthophyll bioavailability in blood and retina.
Protective role of xanthophylls
Authors: ACAR N 
Published: November 2010

AbstractXanthophylls, also known as carotenoids are a group of natural fat‐soluble pigments that are especially abundant in green and yellow‐orange fruits and vegetables. Based on epidemiologic data, it is now evident that carotenoids provide health benefits and particularly in eye diseases. Molecular characterization of retinal carotenoids has shown that there were actually two xanthophylls that are concentrated in macular region, namely lutein and zeaxanthin. Thanks to a strategic position within inner retinal layers, lutein and zeaxanthin are suspected to act as antioxidants in the retina in order to limit oxidative stress that results from metabolism of light. In addition, they are also supposed to absorb blue light that enters the eye prior to its reaching the delicate functional structures including the photoreceptors and the retinal pigment epithelial cells. The aim of this paper is to summarize the knowledge about the biological mechanisms of the protective role of macular xanthophylls.
Potential retinal causes: when and how to investigate
Authors: LEROY BP 
Published: November 2010

Abstract Purpose To describe the retinal conditions that need to be excluded when non‐organic visual loss is suspected, and the investigations required to either confirm or exclude them. Methods A case presentation format will be used to illustrate those conditions which can be discovered using psychophysical and electrophysiological tests as well as special imaging including autofluorescence, infrared and red free imaging and spectral‐domain optical coherence tomography, in patients in whom a non‐organic origin for visual loss is suspected. Results Inherited retinal diseases such as Stargardt macular dystrophy, X‐linked retinoschisis and cone dystrophy as well as Batten disease in their early stages all need to be excluded when visual loss is thought to be non‐organic. In addition, several acquired retinal conditions such as acute acular neuroretinopathy need to be taken into account. visual field testing, ISCEV‐standard full‐field flash electroretinography, pattern electroretinography and visual evoked potentials and specialised imaging techniques contribute significantly to making the correct diagnosis. Conclusion Visual loss in a list of organic conditions may mimic non‐organic visual loss. Functional testing as well as specialised imaging techniques are essential in differentiating true organic from non‐organic visual loss.
Neuro‐ophthalmic considerations and investigations
Authors: BORRUAT FX 
Published: November 2010

AbstractFunctional non‐organic patients can be a challenge to the clinical practitioner. Most likely they will complain of decreased visual acuity and/or of altered visual field. However some patients can complain of oculomotility disturbances (diplopia, oscillopsia, ophthalmoplegia), can present with relative eyelid ptosis, or will exhibit anisocoria. This presentation will highlight certain typical findings of such patients as well as which specific tests can be used in order to detect the non‐organic nature of their complaints.
Boston Type 1 KPro ‐ Multicenter study outcomes
Authors: CIOLINO J , RUDNISKY C , ZERBE B , BELIN M 
Published: November 2010

Abstract Purpose To report indications and outcomes from a multicenter study on the Boston Type 1 keratoprosthesis (Kpro). Methods Kpro data was collected and analyzed at a central collection site. Kpro survival and visual acuity are the main outcomes measured. Results 324 Boston Type 1 Kpro implants (310 patients) from 19 surgeons were included. The average age was 63.2±18.7 years, 47.6% female, 53.0% right eyes. The average number of penetrating keratoplasties (PKP) prior to kPro insertion was 1.9±1.5 (range 1‐8). Pre‐operative diagnosis was divided into 3 different categories: “chemical” (32 eyes), “autoimmune” (39 eyes with either Stevens Johnson Syndrome [SJS] or Ocular Cicatricial Pemphigoid [OCP]), “other” (n=188), and “unknown etiology” (n=65). The mean preoperative logMAR visual acuity was 1.77±0.62 (48.1%; n=156 eyes); 32.4% hand motion and 14.2% of light perception. Average follow‐up time was 16.3±14.6 months (range 0.7 – 59.4). The overall retention rate was 92.0% (n=298). Median survival time (months) was 60.9 (autoimmune), 99.1 (chemical), & 139.3 (other). The median survival time for patients with autoimmune disease was significantly lower than for other diagnoses (p<0.0001). Among eyes with at least 1 year of follow‐up (135 eyes), overall Kpro retention was 90.4%. Mean visual acuity (n=151 eyes) was significantly improved to 0.78±0.58 logMAR units after one year of follow‐up (p<0.0001). The retention rate observed in autoimmune patients (71.8%, p<0.0001) followed by chemical (93.8%), “other” (93.1%) & “unknown” (100%). Conclusion The Boston Type 1 KPro appears to be a viable option for eyes that are not candidates for PKP however caution should be used in eyes with a history of SJS or OCP.
Outcomes of the Boston Keratoprosthesis Type II
Authors: CHODOSH J , PUJARI S , SIDDIQUE S , DOHLMAN C 
Published: November 2010

Abstract Purpose To report the long term outcomes of Boston keratoprosthesis type II implantation in the management of severe ocular surface disease and corneal blindness. Methods A retrospective review of medical records of patients after Boston keratoprosthesis type II implantation at the Massachusetts Eye and Ear Infirmary from January, 1, 2000 through December 31, 2009. Main outcome measures included visual acuity, device retention, and postoperative complications. Results A total of 29 eyes of 26 patients received a Boston keratoprosthesis type II during the study period. Operated patients had corneal blindness due to mucous membrane pemphigoid (51.7%), Stevens Johnson syndrome/toxic epidermal necrolysis (41.4%), or other ocular surface disease (6.9%). Visual acuity after surgery improved to at least 20/200 in 23 eyes (79.3%), and to 20/30 or better in 10 eyes (34.5%). In patients with at least 1 year of follow‐up (n=21), visual acuity of 20/200 or better was maintained in 14 eyes (66.7%). Of these 14 eyes, 6 eyes (42.9%) retained visual acuity of 20/200 or better for more than 5 years. Overall, visual acuity of 20/200 or better was maintained for at least 5 years in 6 of 23 eyes (26.0%). Eyes that failed to improve to 20/200 or lost vision during follow‐up were found to have end stage glaucoma, previous retinal detachment, or age related macular degeneration. Of the total of 29 eyes, 17 devices (58.6%) were retained without extrusion or replacement during a total follow‐up time of 107.9 person‐years. Conclusion The Boston keratoprosthesis type II is a viable option for corneal blindness from severe autoimmune ocular surface diseases. Complications require prompt recognition and management to promote retention of the device and preservation of vision.
Glaucoma considerations in OOKP
Authors: AL RAQQAD N , LEE R , HEROLD J , LIU CSC 
Published: November 2010

Abstract Purpose To study the incidence and prognosis of glaucoma in OOKP eyes and to evaluate methods for detection and treatment of glaucoma following osteo‐odonto‐keratoprosthesis surgery. Methods Retrospective analysis of 49 consecutive patients treated at the national OOKP referral centre in Brighton, UK between November 1996 and September 2009. Data were collected over a 3 month period from December 2009 giving a minimum of 6 months follow up (range: 6 months to 13 years). Data collected include: age at surgery, primary diagnosis, previous surgical procedures (corneal grafts), family history of glaucoma, serial post‐operative cup‐disc ratios and visual fields, glaucoma procedures at any stage, and any OOKP complications. Results A total of 49 patients were included in the study with age ranging from 19 to 88 years (mean 53.5). 14 (28.5%) patients had pre‐existing glaucoma, 25(51%) patients had glaucoma by the end of the study. 12(24.5%) patients developed de novo increase in intraocular pressure. Of the 49 patients, 5 underwent cyclodialysis ,4 had cyclodiode laser treatment, 4 underwent ECP, 5 had a glaucoma tube inserted and 4 had detachment and reattachment of their recti muscles (VRDR ± MRDR). All patients were treated with oral acetozolamide 250mg once daily (except one who is allergic). Sublingual timolol was used in three patients, one of experienced severe side effects and the treatment had to be stopped. Systemic betablockers were used in 3 patients. Conclusion Glaucoma is one of the major complications of osteo‐odonto‐keratoprosthesis. It is difficult to monitor and treat in the OOKP eye. Various treatment modalities have been used. Many patients require long term oral medications and many are not adequately controlled and should have surgical treatment the results of which are not satisfactory. VRDR might represent a safer surgical method to control glaucoma in OOKP eyes.
Biosynthetic corneas ‐ 2 year post human implantation
Authors: FAGERHOLM P , LAGALI N , GRIFFITH M 
Published: November 2010

Abstract Purpose To evaluate biosynthetic corneas as substitutes for donor corneas in humans followed for 2 years. Methods Recombinant human collagen type III was cross‐linked to fabricate corneal substitutes. Following proper study approval, a Phase I study was initiated. 10 patients , scheduled for corneal grafting, were enrolled. Nine had keratoconus and one a deep scar following Pseudomonas keratitis. Lamellar surgery was performed. Six overlying sutures were used to anchor the graft. Topical dexametasone and chloramphenicol was given postoperatively. The sutures were removed after 6 weeks. The patients were followed clinically and evaluated for UCVA, BSCVA and BCLVA. Sensitivity (Cochet‐Bonnet) and Shirmer were tested. Photography, OCT (Visante), topography and IVCM was documented. Results After 3 months all patients had epithelialized and implants were anchored by keratocyte ingrowth. The nerve regeneration after 24 months was lower than normal controls but better than after PKP. The mean BSCVA at 24 months was less than after PKP but BCLCVA came up to par with PKP BSCVA.The corneal thickness was stable between 3 and 24 months at about 400 µm. The Shirmer values were 20 ± 10 mm in operated eyes and 17 ± 8 mm in fellow eyes. At 18 months the sensitivity was 37 mm in operated eyes and 60 mm in fellow eyes. Conclusion Bioengineered corneal substitutes are compatible and promote regeneration of corneal cells. They show stability after 2‐3 months. The results after 2 years will be presented.
Optical characterization of PCO
Authors: VAN DEN BERG TJTP , VAN BREE MCJ 
Published: November 2010

Abstract Purpose Opacification of the posterior capsule (PCO) is known to degrade visual function on both counts: small angle resolution (visual acuity and contrast sensitivity) as well as large angle light scatter (straylight). No studies have been performed to delineate the optical characteristics of PCO as a basis for explanation of these visual function defects. Methods Preparations of IOL‐capsule combinations were harvested from donor eyes after removal of the cornea. They were mounted submersed in saline in an optical set‐up, used earlier to characterize the optics of the crystalline lens (van den Berg et al. VR 1999). Illuminated by monochromatic beams of light, the light spreading originating from different areas of the preparation was measured as function of wavelength for different angles. Based on physical optics theory, the optical characteristics of PCO was derived. The data will be presented translated into the straylight parameter, directly comparable to the figures obtained in vivo using the clinical straylight meter (C‐Quant from Oculus). Results Twenty successful preparations were harvested with at least some PCO areas. PCO grades varied from severe to slight. Two physically different types of light spreading could be discriminated. One type corresponding to pearl‐like appearance, characterized by little wavelenght dependence and smal angle dominance. The other type corresponding to diffuse scatter, characterized by strong wavelength dependence and large angle dominance. The scatter values measured corresponded to clinical measures of straylight in PCO patients using the C‐Quant. Conclusion The light scattering characteristics of PCO show two types of optical disturbance: a refractile type, probably dominated by pearl‐like structures, and a diffuse type of scattering of yet unclear origin.
Capsular peeling in premium IOLs to improve visual outcome
Authors: TASSIGNON MJ , ROZEMA J 
Published: November 2010

Abstract Purpose To demonstrate that capsular peeling may improve quality of vision of patients who experienced reduced vision in the short postoperative period after premium IOL implantation due to decentration of the IOL secondary to PCO. Methods Patients who were refered to our centre because of unsatisfactory quality of vision after premium IOL implantation (more specifically after multifocal IOLs) were scheduled for surgery aiming at peeling the capsular bag. The conditions which the patient needed to meet were: increased higher order aberrations, evidence of tilt or decentration of the IOL, important fibrotic proliferation in the capsular bag, no YAG laser capsulotomy performed. Results After capsular peeling, it was possible to demonstrate that the quality of vision of the patient improved, the higher order aberrations reduced and it was often unnecessary to explant the premium IOL. Although there was an obvious improvement of the quality of vision, this improvement still did not reached the high standard of vision as measured after monofcal IOLs. Conclusion This paper shows how important the role is of PCO on quality of vision. Because contrast sensitivity is already reduced after premium IOL implantation, patients will be very sensitive to an additional reduction as it appears after PCO.
General principles of autoinflammation and autoimmunity
Authors: WILLERMAIN F 
Published: November 2010

Abstract Purpose In this talk, the definition and the molecular mechanisms of autoinflammation and autoimmunity will be introduced. Methods Defense against invading microorganisms is one of the main challenges of life. Very early in the evolution, a series of germline‐encoded protein capable to detect pathogen associated molecular patterns (PAMP) have evolved. PAMPs include toll‐like receptors, NOD like receptors and C‐type lectin. Their activation converges to the rapid stimulation of proinflammatory pathways. Results PAMPs are at the basis of the innate immune response which represent the first line of defense and will shape the nature of the adaptive immune system. The latter is mediated by clonal selection and expansion of antigen specific T and B lymphocytes. It is now well described that dysregulations of those two arms of the immune system are associated with distinct clinical diseases. Conclusion Various anomalies of the innate immune system have been found in a series of disease grouped under the name autoinflammatory syndromes. This term highlight the distinction between those diseases and classical autoimmune diseases, characterized by an abnormal adaptive immune response with the presence of autoantibodies and/or autoreactive T cells.
An introduction to autoinflammatory syndromes
Authors: BODAGHI B 
Published: November 2010

AbstractTo define the spectrum and pathophysiology of autoinflammatory syndromes. This term has been proposed to describe a new group of diseases characterized by attacks of seemingly unprovoked inflammation in the absence of pathogens, without significant levels of autoantibodies and autoreactive T cells. Hereditary periodic fever syndrome, Crohn's disease, Blau syndrome, Chronic infantile neurologic cutaneous and articular syndrome and Muckle‐Wells syndrome are examples of autoinflammatory conditions characterized by recurrent attacks of inflammation without any association with auto‐antigens. The study of autoinflammatory diseases has progressed from genetics to definition of the functional defects. Although a direct association between defective innate immune responses to bacterial components and these diseases has not been established yet, this hypothesis remains highly plausible. Mutations in genes encoding the tumour necrosis factor (TNF) receptor and pyrin superfamilies of molecules may induce persistence of leukocytes that would ordinarily undergo apoptosis with further amplification of inflammatory stimuli. The use of biologics may control some of these conditions.
Infectious and non infectious triggers in non‐infectious uveitis
Authors: WILDNER G 
Published: November 2010

Abstract Purpose The induction of autoimmune uveitis is difficult to explain with respect to the immune privileged status of the eye. The intact BRB can only be passed by already activated leukocytes, which should normally be ignorant to the sequestered intraocular antigens. Antigenic mimicry of retinal autoantigens by environmental proteins could explain extraocular activation of effector T cells. Methods We have previously demonstrated antigenic mimicry of a peptide from retinal S‐Antigen and peptides from rotavirus (Rota) and bovine milk casein (Cas). Both, Rota and Cas, induce T cell lines cross‐reactive with retinal S‐Ag peptide as well as experimental autoimmune uveitis in rats. Patients with uveitis have increased antibody and T cell responses to the mimicry peptides as well as to the S‐Ag peptide compared to healthy donors. Accordingly, Infection with rotavirus or any gastrointestinal pathogen with concomitant ingestion of bovine milk products could induce an immune response in the gastrointestinal tract that is cross‐reactive with ocular autoantigens and lead to induction of autoimmunity in the eye. Results Uveitis as a well known adverse effect after BCG (Bacille Calmette Guerin) treatment might also be the result of antigenic mimicry. We have shown T cell responses to PPD from M. tuberculosis and the retinal autoantigens S‐Ag, IRBP and CRALBP from a patient who had developed granulomatous uveitis after BCG application for bladder carcinoma. Data base searches revealed a number of amino acid sequence homologies between proteins from mycobacteria and retinal autoantigens, suggesting antigenic mimicry. These findings might as well be an explanation for the occurrence of uveitis in connection with M. tuberculosis infection, even when no mycobacteria are detectable in the eye.
Integrating the interplay between innate and adaptive immunity in the development of non infectious uveitis
Authors: DICK AD , NICHOLSON LB 
Published: November 2010

Abstract Purpose Whilst uveitis is a CD4 T cell mediated disease, there remains both the interplay with innate immunity at both the affector (initiation) of disease and moreso the phenotype and severity of the effector stages of disease. Methods The talk will describe the experimental evidence to describe the interplay of T cells and myeloid cells within the retina during intraocular inflammation. Results TNF, whilst a pleitropic cytokine, has significant influence of the disease phenotype and disease course we observe experimentally. TNF is requred for activation of myeloid cells (macrophages)when in presence of Th1 CD4 T cells, which results in contribution to tissue damage but also controlling T cell proliferation in situ via generation of myeloid suppressor cells. Moreover, the role of resident myeloid cells (microglia) is to down‐regulate the immune response. The role of myeloid cells in the healing response and angiogenesis will also be discussed with respect to T cell responses. Conclusion Myeloid cells are actively conditioned by their envirnoment (cognate and soluble factors) to respond accordingly. As such the regulation of immune response and healing that follows is dictated by the innate system in response to changes in local and systemic conditions.