bluuebunny/crossref_metadata_2025_split huggingface dataset:
Customer Differentiation with Shipping as an Ancillary Service? Free Service, Prioritization, and Strategic Delay
Authors: Sainathan Arvind 
Published: September 2017

ABSTRACTA service provider/retailer offers ancillary service (e.g., shipping by an online retailer) to two types of customers, impatient and patient, who may be heterogeneous both in their delay sensitivities and service valuations. She can use prioritization and/or strategic delay to differentiate them by offering two service classes and charging different prices, potentially resulting in a split in which a single customer type selects both the classes. Her objective is to minimize cost while satisfying individual rationality and incentive compatibility conditions. We characterize the optimal solutions under both exogenous and endogenous capacities. We examine the conditions under which the following strategically important features of service delivery are optimal, and relate them to practical scenarios: (i) free service, (ii) single/differentiated service, (iii) split of customers, and (iv) strategic delay. We find that the presence of these features depends on (i) whether the retailer has limited or sufficient capacity and (ii) whether she sells fashion goods or staple products. A typical explanation for offering free service is that it increases demand from customers. We make an operational case for it by showing that even if demand does not change, free service is still optimal under some scenarios.
Dravet syndrome and its mimics: Beyond SCN1A
Authors: Steel Dora , Symonds Joseph D. , Zuberi Sameer M. , Brunklaus Andreas 
Published: September 2017

SummaryObjectiveDravet syndrome (DS) is a severe developmental and epileptic encephalopathy characterized by the onset of prolonged febrile and afebrile seizures in infancy, and evolving to drug‐resistant epilepsy with accompanying cognitive, behavioral, and motor impairment. Most cases are now known to be caused by pathogenic variants in the sodium channel gene SCN1A, but several other genes have also been implicated. This review examines current understanding of the role of non‐SCN1A genes in DS, and what is known about phenotypic similarities and differences. We discuss whether these are best thought of as minority causes of DS, or as similar but distinct conditions.MethodsBased on a review of literature, a list of genes linked to DS was compiled and PubMed was searched for reports of DS‐like phenotypes arising from variants in each. Online Mendelian Inheritance in Man (OMIM) was used to identify further reports relevant to each gene.ResultsGenes that have been reported to cause DS‐like phenotypes include SCN2A, SCN8A, SCN9A, SCN1B, PCDH19, GABRA1, GABRG2, STXBP1, HCN1, CHD2, and KCNA2. Many of these genes, however, appear to be associated with their own, different, clinical picture. Other candidate genes for DS have been reported, but there is currently an insufficient body of literature to support their causative role.SignificanceAlthough most cases of DS arise from SCN1A variants, numerous other genes cause encephalopathies that are clinically similar. Increasingly, a tendency is noted to define newly described epileptic disorders primarily in genetic terms, with clinical features being linked to genotypes. As genetic diagnosis becomes more readily available, its potential to guide pathophysiologic understanding and therapeutic strategy cannot be ignored. Clinical assessment remains essential; the challenge now is to develop a gene‐based taxonomy that complements traditional syndromic classifications, allowing elements of both to inform new approaches to treatment.
Popper associated maculopathy – case report and literary synthesis
Authors: Murphy R. , James M. , Cullinane A. 
Published: September 2017

PurposePrevalence of Alkyl Nitrate, or ‘Poppers’ abuse has remained high since its putative birth in the urban disco scene of the 1970's, with UK figures from 2014 suggesting a 9.1% adult lifetime use rate. However, an associated retinal toxicity is a new and emerging phenomenon due to the recent change of its main compound and continued popular recreational use. Here, we describe a case of Alkyl Nitrate associated maculopathy.MethodsCase report with literary synthesis.ResultsA 44‐year‐old Caucasian male with no previous ophthalmic history presented to our eye casualty department with bilateral central vision blurring following repeated inhalation of Alkyl nitrates. Drug use was believed to have occurred twice daily by inhalation over an interrupted 6‐week period. Best‐corrected visual acuity at presentation was 6/6‐1 in the right eye and 6/9+1 in the left. Fundal photography reveals subtle yellow foveal spots bilaterally with an otherwise normal peripheral retina and optic disc. Optical coherence topography demonstrates marked disruption of the photoreceptor inner segment / outer segment junction. Fundus Fluorescein Angiography exhibits a subtle foveal hyper‐fluorescence bilaterally. Multifocal ERG displayed attenuated responses from the foveal and parafoveal segments of the left eye with blunting of the foveal peak. A synthesis of current literature and various hypotheses to date is subsequently discussed.ConclusionsThe rarity of this phenomenon, along with tendencies of reluctant disclosure of substance abuse behaviours, can lead to diagnostic difficulties. When faced with bilateral painless central blurring, in otherwise healthy, young individuals, physicians should obtain a detailed history of possible substance abuse.
Prospective comparison of global visual field indices and cluster progression in glaucoma and their relationship to structural changes
Authors: Bono V. , Normando E.M. , Davis B. , Cordeiro M.F. 
Published: September 2017

PurposeGlaucoma diagnosis and follow up of progression is often based on structural and functional assessments. This study aimed to assess clustered progression in comparison to global indices and structural measures from HRT and OCT.Methods16 eyes of OHT and glaucoma patients with a minimum of 5 visual fields (HFA II i 24‐2) over a year were assessed prospectively. MD and VFI rates of progression were used for trend analysis. Linear regression of clusters defined by the Glaucoma Hemifield Test (GHT) was performed based on the mean threshold in each cluster. Rim Area (HRT 3) and mean parapapillary RNFL(SD‐OCT) analysis were assessed and correlated with functional clustered measures. Rates of progression were flagged as statistically significant if the gradients over time were negative with p<0.05.ResultsCluster analysis showed significant progression: 11 of 16 eyes in at least 1 cluster (68.75%) with an average rate of progression of ‐2.18 ± 2.2 dB/year. RNFL thinning was found in 12 of 16 eyes with an average rate of progression of 2.58 ± 1.43 μm/year. 7 out 16 eyes were progressing at HRT RA (43.75%) while 5 of 16 and 6 of 16 were progressing at VF VFI (31.25%) and VF MD (37.5%) respectively. The agreement between GHT Cluster and VF VFI and VF MD was 0.34 and 0.2, respectively. The best agreement was found between GHT Cluster and OCT RNFL (k= 0.61).ConclusionsVisual field clusters well detected spatial locations of sensitivity loss showing greater sensitivity than global indices (MD; VFI) and better concordance with structural changes. This suggests GHT clusters to be a sensitive method for the early identification of glaucomatous visual field loss.
Classification of uveitis
Authors: Markomichelakis N. 
Published: September 2017

SummaryClassification of uveitis is important for diagnostic reasons. The first step in developing a differential diagnosis for patients with an intraocular inflammation is to classify the uveitis in detail. Proper classification is also essential if one is to avoid confusion and misinterpretation among multiple ophthalmologists caring for a given patient. Different types of classification are used: The anatomic classification should not be confused or overlap with the etiologic classification. Both classifications are required and important, but they are distinct and different.
Measurement of retinal blood flow using Doppler OCT in diabetic retinopathy
Authors: Bek T. 
Published: September 2017

SummaryPurposeTo determine factors of importance for quantitating retinal blood flow in patients with diabetic retinopathy using bi‐directional Doppler OCT.MethodsRetinal blood flow was measured by bi‐directional Doppler OCT in 20 diabetic patients with different diabetes type, age and retinopathy stage.ResultsIt appeared that age above 40 years was a significant limiting factor for obtaining suitable signals to measure retinal blood flow, which was due to lens opacities and insufficient mydriasis. Quantitative measures of blood flow in the studied patients below the age of 40 years will be reported.ConclusionBi‐directional Doppler OCT offers a new principle for quantitating retinal blood flow, but the principle has not yet been fully developed to be suitable for routine clinical practise.
Retinal oximetry in central retinal vein occlusion
Authors: Jeppesen S.K. , Bek T. 
Published: September 2017

PurposeTo determine the significance of retinal oxygen saturation for visual acuity at diagnosis and after anti‐VEGF treatment of central retinal vein occlusion (CRVO).MethodsRetinal oximetry was performed in 91 patients with CRVO at the Department of Ophthalmology, Aarhus University Hospital, and the predictive value of the retinal oxygen saturation in larger retinal vessels for the visual prognosis after anti‐VEGF medication was studied.ResultsAt baseline the oxygen saturation in larger retinal vessels was significantly increased in arterioles and decreased in venules in the affected eye. Best corrected visual acuity (BCVA) showed a significant negative correlation with the oxygen saturation in retinal arterioles and a positive correlation with the oxygen saturation in retinal venules. Multiple linear regression showed that BCVA, but not oxygen saturations, contributed significantly to predicting visual outcome after treatment.ConclusionMeasurement of retinal oxygen saturation may help understanding haemodynamic and visual changes in the acute stages of CRVO. However, retinal oximetry cannot replace measures of retinal function as a predictive parameter for the visual outcome in CRVO after intravitreal anti‐VEGF therapy.
Assessment of retinal blood flow using Laser Speckle Flowgraphy
Authors: Schmidl D. , Witkowska K. , Luft N. , Bolz M. , Fondi K. , Bata A. , Wozniak P. , Werkmeister R. , Garhofer G. , Schmetterer L. 
Published: September 2017

SummaryLaser speckle flowgraphy (LSFG) is a promising technique for the two‐dimensional, non‐invasive assessment of ocular blood flow. We set out to investigate the use of this technique in healthy Caucasians. First, we evaluated the validity of retinal perfusion measurements by direct comparison with dual‐beam Doppler optical coherence tomography (D‐OCT). Relative flow volume (RFV), the main output parameter of LSFG, was in significant agreement with absolute blood flow measurements as obtained from D‐OCT in arteries (r = 0.69, P = 0.001) and veins (r = 0.74, P < 0.001). We also found that most of LSFG‐derived parameters were age‐dependent in a healthy population. In addition, retinal blood flow autoregulation during isometric exercise was studied using LSFG. Retinal blood flow started to increase significantly at ocular perfusion pressure levels that were 45% over baseline. These findings are comparable to data obtained in previous studies. The presented results show that LSFG can be applied to study ocular blood flow in healthy Caucasian subjects. The technique may also be used in patients with ocular diseases associated with vascular dysregulation such as glaucoma, age‐related macular degeneration or diabetic retinopathy in the future.
Bilateral quantification of vascular density in diabetic patients using optical coherence tomography angiography
Authors: Czako C. , Ecsedy M. , Récsán Z. , Szepessy Z. , Resch M. , Borbándy Á. , Tátrai E. , Sándor G. , Horváth H. , Zsolt Nagy Z. , Kovács I. 
Published: September 2017

PurposeTo measure capillary vessel density (VD) using optical coherence tomography angiography (OCT‐A) in both eyes of diabetic patients and to evaluate its correlation with systemic risk factors.MethodsA total of 103 eyes of 61 diabetic patients underwent OCT‐A imaging (RTVue‐XR Avanti; Optovue, Fremont, CA, USA). The control group included 92 eyes of 46 individuals without diabetes mellitus. In diabetic patients the duration of diabetes, insulin therapy, blood pressure, HbA1C, dyslipidemia and the presence of diabetic retinopathy was recorded. VD was examined in the central macula with a radius of 3 mm. The effect of risk factors on VD was assessed bilaterally using multivariable regression analysis.ResultsVessel density was significantly decreased in diabetic patients compared to controls (47.26 vs. 50.88%; p < 0.001). In control subjects, VD significantly decreased with age (r = −0.54; p < 0.001), while in diabetic patients VD was associated only with the duration of diabetes among the risk factors (r = ‐0.15/year, p = 0.03). VD was also significantly decreased in diabetic patients without clinically detectable diabetic retinopathy compared to control subjects (48.19 vs. 50.88%, p < 0.001). Between‐eye difference in VD were significantly higher in diabetic patients with retinopathy (4.46±2.95%) and without retinopathy (4.19 ± 2.40%) in comparison to the controls (2.74 ± 2.24%; p = 0.02 for both comparisons).ConclusionsSignificantly decreased retinal vessel density and increased between‐eye asymmetry can be measured in diabetic patients compared to control subjects even before the presence of clinically detectable diabetic retinopathy. While in control subjects vessel density decrease with aging, in diabetic patients both vessel density and between‐eye asymmetry are significantly associated with the duration of diabetes.
Differential diagnosis of cystoid macular edema by optic disc thickness in optical coherence tomography
Authors: Cardigos J. , Crisostomo S. , Basilio A. , Costa L. , Carvalho B. , Vieira L. , Flores R. 
Published: September 2017

PurposeThe differential diagnosis between Irvine‐Gass Syndrome (IGS) and Diabetic Macular Edema (DME) is currently established by angiography, which is an invasive and time‐consuming tool. The aim of this study is to evaluate differences in optic disc's nerve fiber layer thickness (RNFL) in patients with macular edema associated with IGS and Diabetes Mellitus (DM).MethodsRetrospective case control study of 28 eyes of 28 patients with macular edema divided into 2 groups. Group 1 included 14 eyes with IGS diagnosed by clinical and angiographic criteria and group 2 included 14 eyes with cystoid diabetic macular edema (DME) without previous ocular surgery. A control group (group 3) of 14 eyes of 14 healthy patients paired by age and gender were also included. Ophthalmologic examinations included visual acuity (VA) measurements, slit‐lamp examination, Goldmann applanation tonometry, Spectral‐Domain Optic Coherence Tomography (SD‐OCT) and fluorescein angiography.ResultsForty two patients (21 females and 21 males), with a mean age of 66.57 ± 10.80 years were included in this study. Optic disc nasal and temporal RNFL thicknesses as well as central macular thickness were significantly higher in group 1 and 2 compared with control group (p < 0.05). Nasal RNFL thickness was higher in IGS than in patients with DME (p < 0.001). To detect IGS (Group 1), comparative to DME (Group 2), using Nasal RNFL thickness, the area under the curve was 0.842 p < 0.002. A cut‐off criterion of 82.50 μm resulted in 92.9% sensitivity and 71.4% specificity.ConclusionsOptic disc SD‐ OCT imaging might be a useful diagnostic tool to differentiate IGS from DME.
Intraconal hybrid neurofibroma ‐ schwannoma of the orbit
Authors: Verhelst E. , Lauwers N. , Siozopoulou V. , de Keizer R.W.J. , De Groot V. 
Published: September 2017

PurposeAmong peripheral nerve sheet tumors, an orbital hybrid neurofibroma‐schwannoma has been described twice. We describe a third case of orbital tumor with features of both a neurofibroma and a schwannoma.MethodsCase report. A 39 year old man presents with diplopia and decreased vision (0.6) in the left eye. He has a proptosis of 7 mm, with mechanical restriction of elevation and horizontal eye movements. MRI shows a well‐defined lesion (2,5 x 1,9 x 2,3 cm) in the superotemporal intraconal space, displacing the globe, optic nerve and superior and lateral rectus muscles. The tumor demonstrates heterogeneous contrast enhancement, and is diagnosed as a probable cavernous hemangioma. Through a transconjunctival approach a red‐blueish lesion was found with a thin capsule and without lateral adhesions. The tumor could be extracted in 2 parts and consisted of a yellow/white coherent soft mass.ResultsPatient had complete recovery of visual acuity, and only diplopia in extreme lateral gaze. The pathology revealed a tumor composed of small spindle cells arranged in fascicles. The cells showed no atypia and there was no mitotic activity. There were also less cellular areas with a myxoid component. At places we could recognize a fibrous capsule surrounding the tumor. The tumor cells were strongly and diffuse positive with S100. In a few enclosed axons there was focal reactivity with neurofilament. The image is that of a neural tumor with features of both a neurofibroma and a Schwannoma.ConclusionsThis is the third reported orbital hybrid neurofibroma‐schwannoma. Although this type of tumor elsewhere in the body is often associated with schwannomatosis and neurofibromatosis, our patient did not show signs of systemic involvement.
The advantages of serological tests of blood and tears for the diagnosis and the follow up of corneal rickettsiosis
Authors: Benabderrahim K. , Feki J. , Khairallah M. 
Published: September 2017

PurposeSerological tests were rarely performed in the first intention in the diagnosis of corneal rickettsiosis in patients from an endemic area. The purpose is to elucidate the advantages of serological tests for rickettsiosis in the atypical cases of corneal infectionsMethodsCase series study included 03 patients. immunofluorescent‐antibody tests were performed to detect rickettsiosis in the blood and in the tearsResults2 patients with atypical disciform keratitis, 1 patient with panuveitis with corneal involvement. Rickettsiosis was suspected because of skin palpebral lesions in 2 patients and in chronic uveitis in the 3rd patients. Serological tests were positive in the blood and in the tears. Treatment with tetracyclin was effective in the three cases without use of corticotherapy. In the follow up, good evolution of corneal signs and negative serological tests were noted.ConclusionsRickettsiosis should be considered in the etiology of atypical cases of corneal infection and serological blood and tear testing should be performed firstly in patients from an endemic area.
Retinal vasculitis
Authors: Bodaghi B. 
Published: September 2017

SummaryNearly all etiologies of intermediate, posterior or panuveitis may be associated with retinal vasculitis. The clinical analysis of different inflammatory markers is the first step for diagnostic orientation and further work‐up. Different cases will be discussed in an interactive manner in order to highlight the vascular type of involvement, associated vitreous inflammation, macular edema, retinal or choroidal lesions such as granuloma or ischemia. A systemic autoimmune, autoinflammatory or infectious disease have also to be taken into consideration. Therapeutic options remain significantly large, ranging from close monitoring to corticosteroids, different types of immunosuppressors, biolgics, antimicrobials, anti‐VEGFs, laser photocoagulation and rarely vitrectomy. Prognosis is variable based on etiology, diagnostic delay and therapeutic efficacy.
Human case
Authors: Sergouniotis P. , Ellingford J. , Hall G. , Ramsden S. , Biswas S. , Ashworth J. , Black G. 
Published: September 2017

SummaryInherited eye disease cases will be presented with an emphasis on conditions with readily recognisable features that aid precise diagnosis. Cases of monogenic disorders mimicking common, multifactorial disease will also be shown. The value of genomic testing will be highlighted and the role of investigations to exclude/identify extraocular involvement will be discussed.
The supportive role of interferon‐γ in retinal differentiation of mesenchymal stem cells
Authors: Hermankova B. , Kossl J. , Javorkova E. , Bohacova P. , Hajkova M. , Zajicova A. , Krulova M. , Holan V. 
Published: September 2017

PurposeRetinal disorders represent a serious health problem causing a decreased quality of vision or even blindness. There are currently no effective treatment protocols for these disorders. The promising approach for the treatment of retinal disorders is stem cell‐based therapy. Mesenchymal stem cells (MSCs) are a perspective candidate due to their possibility to migrate to the site of injury, differentiate into multiple cell types and produce a number of trophic factors. In this study we analysed the potential of murine bone marrow‐derived MSCs to differentiate into cells expressing retinal markers and tested the possibility to express neurotrophic factors by differentiated MSCs.MethodsFlow cytometry was use to characterize the phenotypic markers of murine MSCs isolated from bone marrow. The retinal extract were prepared by homogenization of posterior segments of the mouse eyes and the supernatants were prepared by stimulation of spleen cells with Concanavalin A. MSCs were cultured with retinal extract and supernatants simulating the environment of retinal damaged for 7 days. The expression of genes for retinal markers and growth factors by MSCs was detected using real‐time PCR.ResultsMSCs cultured with retinal extract and supernatant differentiated to the cells expressing retinal cell markers. To identify a supportive molecule in the supernatant from activated spleen cells, MSCs were cultured with retinal extract in the presence of various T‐cell cytokines. The expression of retinal markers was enhanced only in the presence of IFN‐γ, and the supportive role of spleen cell supernatants was abrogated with the neutralization antibody anti‐IFN‐γ.ConclusionsThe results show the supportive role of IFN‐γ in differentiation of MSCs to the cells expressing retinal cell markers and the enhanced ability of differentiated cells to express growth factors.
Forgetting malignancies
Authors: Sen H.N. 
Published: September 2017

SummaryMasquerade syndromes are a rare group of neoplastic or non‐neoplastic disorders that present as uveitis. Approximately 40% of all uveitis cases remain idiopathic despite exhaustive clinical and laboratory investigations. The consequence of missing a malignant masquerade syndrome can be devastating. Among uveitis patients older than 60 years, malignancy constitutes 1.5‐5% of all cases. Among uveitis patients of all ages malignant masquerade syndromes constitute only 2‐3% of cases in tertiary uveitis centers. There are some distinguishing characteristics between uveitis patients and neoplastic masquerade patients: patients with neoplastic masquerade often present at an older age, have unilateral disease and posterior segment involvement and are more likely to be Caucasian. Diagnosis of malignancies in the eye can be difficult but important and clinical features at presentation can help raise suspicion. Early diagnosis and intervention not only saves vision and improve quality of life but it can also be life‐saving.