Evaluation of early and late presentation of patients with ocular mucous membrane pemphigoid to two major tertiary referral hospitals in the United Kingdom

PURPOSE: Ocular mucous membrane pemphigoid (OcMMP) is a sight-threatening autoimmune disease in which referral to specialists units for further management is a common practise. This study aims to describe referral patterns, disease phenotype and management strategies in patients who present with either early or established disease to two large tertiary care hospitals in the United Kingdom.\ud \ud PATIENTS AND METHODS: In all, 54 consecutive patients with a documented history of OcMMP were followed for 24 months. Two groups were defined: (i) early-onset disease (EOD:<3 years, n=26, 51 eyes) and (ii) established disease (EstD:>5 years, n=24, 48 eyes). Data were captured at first clinic visit, and at 12 and 24 months follow-up. Information regarding duration, activity and stage of disease, visual acuity (VA), therapeutic strategies and clinical outcome were analysed.\ud \ud RESULTS: Patients with EOD were younger and had more severe conjunctival inflammation (76% of inflamed eyes) than the EstD group, who had poorer VA (26.7%=VA<3/60, P<0.01) and more advanced disease. Although 40% of patients were on existing immunosuppression, 48% required initiation or switch to more potent immunotherapy. In all, 28% (14) were referred back to the originating hospitals for continued care. Although inflammation had resolved in 78% (60/77) at 12 months, persistence of inflammation and progression did not differ between the two phenotypes. Importantly, 42% demonstrated disease progression in the absence of clinically detectable inflammation.\ud \ud CONCLUSIONS: These data highlight that irrespective of OcMMP phenotype, initiation or escalation of potent immunosuppression is required at tertiary hospitals. Moreover, the conjunctival scarring progresses even when the eye remains clinically quiescent. Early referral to tertiary centres is recommended to optimise immunosuppression and limit long-term ocular damage.\ud \u

The management of acute anterior uveitis complicating spondyloarthritis: Present and future

Spondyloarthropathies (SpA) encompass a group of chronic inflammatory diseases sharing common genetic and clinical features, including the association with HLA-B27 antigen, the involvement of both the axial and the peripheral skeleton, the presence of dactylitis, enthesitis, and typical extra-articular manifestations such as psoriasis, inflammatory bowel disease, and acute anterior uveitis (AAU). The latter is commonly reported as a noninfectious acute inflammation of the anterior uveal tract and its adjacent structures. AAU may affect more than 20% of SpA patients representing the most common extra-articular manifestation of the disease. Considering the potential consequences of untreated AAU, early diagnosis and aggressive treatment are crucial to avoid complications of remittent or chronic eye inflammation, such as visual loss and blindness. The management of SpA has dramatically improved over the last decades due to the development of new treat-to-target strategies and to the introduction of biologic disease modifying antirheumatic drugs (bDMARDs), particularly tumor necrosis factor alpha inhibitors (TNFis), currently used for the treatment of nonresponder patients to conventional synthetic agents. Along with the improvement of musculoskeletal features of SpA, bDMARDs provided an additional effect also in the management of AAU in those patients who are failures to topical and systemic conventional therapies. Nowadays, five TNFis, one interleukin-17, and one interleukin 12/23 blocker are licensed for the treatment of SpA, with different proven efficacy in preventing and treating ocular involvement. The aim of this review is to summarize the current options and to analyze the future perspectives for the management of SpA-associated AAU

Refining computer-assisted SEEG planning with spatial priors – A novel comparison of implantation strategies across adult and paediatric centres

Objectives: Computer-assisted planning (CAP) allows faster SEEG planning and improves grey matter sampling, orthogonal drilling angles to the skull, reduces risk scores and minimises intracerebral electrode length. Incorporating prior SEEG trajectories enhances CAP planning, refining output with centre-specific practices. This study significantly expands on the previous work, compares priors libraries between two centres, and describes differences between SEEG in adults and children in these centres. Methods: 98 adults and 61 children who underwent SEEG implantation as part of epilepsy surgery investigations were included. Priors libraries were created for each population, clustered by target regions and subdivided by cortical approaches. The libraries were coregistered and quantitatively and qualitatively compared. Results: The average number of implanted electrodes per patient was higher in paediatric patients than adults (13.6 vs 8.0). Paediatric implantations focused more on the insula than adult implantations (38.0 % vs 13.5 %), with similar proportions of electrodes implanted in the temporal and parietal lobes, and a higher proportion of adult electrodes in the frontal and orbitofrontal regions (40.6 % vs 24.0 %). Correspondence between the priors libraries was high. We present an example of a complex insular implantation planned with paediatric spatial priors and illustrate resultant SEEG recordings. Discussion: The use of centre-specific spatial priors allows the incorporation of surgeon-specific and unit-specific preferences into automated planning. We compare implantation styles between a paediatric and an adult centre, discussing similarities and differences. This tool allows centres to compare practice and represents an effective way to analyse implantation strategies that is agnostic to method of implantation

Refining computer-assisted SEEG planning with spatial priors – A novel comparison of implantation strategies across adult and paediatric centres

ObjectivesComputer-assisted planning (CAP) allows faster SEEG planning and improves grey matter sampling, orthogonal drilling angles to the skull, reduces risk scores and minimises intracerebral electrode length. Incorporating prior SEEG trajectories enhances CAP planning, refining output with centre-specific practices. This study significantly expands on the previous work, compares priors libraries between two centres, and describes differences between SEEG in adults and children in these centres. Methods98 adults and 61 children who underwent SEEG implantation as part of epilepsy surgery investigations were included. Priors libraries were created for each population, clustered by target regions and subdivided by cortical approaches. The libraries were coregistered and quantitatively and qualitatively compared. ResultsThe average number of implanted electrodes per patient was higher in paediatric patients than adults (13.6 vs 8.0). Paediatric implantations focused more on the insula than adult implantations (38.0 % vs 13.5 %), with similar proportions of electrodes implanted in the temporal and parietal lobes, and a higher proportion of adult electrodes in the frontal and orbitofrontal regions (40.6 % vs 24.0 %). Correspondence between the priors libraries was high. We present an example of a complex insular implantation planned with paediatric spatial priors and illustrate resultant SEEG recordings. DiscussionThe use of centre-specific spatial priors allows the incorporation of surgeon-specific and unit-specific preferences into automated planning. We compare implantation styles between a paediatric and an adult centre, discussing similarities and differences. This tool allows centres to compare practice and represents an effective way to analyse implantation strategies that is agnostic to method of implantation.</p

Anti-CD 20 monoclonal antibody (rituximab) treatment for inflammatory ocular diseases

Rituximab is a monoclonal antibody directed against the CD20 antigen expressed on B cells and widely used in the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. There is a growing amount of literature which suggests that rituximab may be useful for inflammatory ocular diseases and intraocular lymphoma. Few cases have been reported on treatment of refractory scleritis, peripherative ulcerative keratitis, uveitis and ocular surface inflammatory disorders. Rituximab may be effective in the treatment of ocular inflammatory diseases in particular the most aggressive, recalcitrant and sight-threatening forms of inflammation such as uveitis associated to juvenile idiopathic arthritis. We review the literature covering the use of Rituximab in these conditions and report our results on the efficacy of Rituximab in the treatment of 8 children with very severe and long-standing uveitis who failed to respond to one or more TNF blockers. Our patients showed improvement in activity of uveitis, reduction of concomitant corticosteroids and immunosuppressants after a mean follow-up time of 14.87 months on rituximab. No serious adverse events were encountered in our treated patients. Although further studies are needed for assessing the efficacy of rituximab and the exact dosing regimen, rituximab may be considered as a treatment alternative in patients with the most aggressive forms of inflammatory ocular diseases who fail to respond to conventional and anti-TNF immunosuppressive agent

The depositional record of the Odyssea drift (Ross Sea, Antarctica)

The Ross Sea is one of the major areas for Antarctic Bottom Water formation (the Ross Sea Bottom Water, RSBW), representing the densest ocean water mass, filling the deepest ocean basins connected to the southern ocean. Peri- odic refill of the RSBW occurs through formation of dense, cold and saline water masses (brine) forming on the shelf at the Ross Sea permanent polynya by freezing and salt rejection (high-salinity shelf water, HSSW). The HSSW periodically overspills the shelf area and descends along the slope. This mechanism represents the engine of the global ocean circulation regulating the climate. The Hillary Canyon, crossing the Ross Sea continental slope, represents one of the main conducts through which the HSSW descends the slope to reach the deeper ocean. On its western levee, there is a mounded depocen- tre that was mapped and ground-sampled during the Italian ITRS17-ODYSSEA expedition on board the RV OGS-Explora (January-February 2017). Geophysical data allowed interpreting such feature as a sediment drift (ODYSSEA Drift), generated by along-slope, contour currents sediment transport and accumulation through sev- eral hundred-thousands years. It was inferred that contour currents transported and deposited the sediments that descent the Hillary Canyon by means of the HSSW. Therefore, the depositional sequence of the ODYSSEA Drift potentially contains the record of the variability of HSSW formation, the along slope current intensity in associa- tion to climate change, and the interplay between the two bottom currents. A multidisciplinary investigation was applied to six gravity cores collected in the proximal and distal area of the ODYSSEA Drift. The cores were analysed to reconstruct the age model combining AMS radiocarbon dating on foraminifera tests, biostratigraphy, and the sediment palaeomagnetic record; the sediment physical properties (wet bulk density, water content and grain size); and compositional characteristics (XRF core scan and geochemistry). Three main lithofacies were distinguished and associated to depositional processes and climatic conditions: 1) finely laminated and bioturbated sediments characterized by a relatively high Ca content with common presence of biogenic component. Such facies was associated to contour current deposition during relatively warm conditions. 2) Bioturbated sediments with abundant, sparse and/or layered Ice Rafted Debris, and high Ca content. The onset of this facies is characterized by a prominent Mn peak that was associated to bottom ocean oxygenation through ice sheet melting/decay. 3) Laminated, barren sediments associated to steady strong bottom currents under harsh climate conditions. Further preliminary data interpretations are discussed

Reappraising prediction of surgical complexity of non-functioning pituitary adenomas after transsphenoidal surgery: the modified TRANSSPHER grade

Purpose: Prognostication of surgical complexity is crucial for optimizing decision-making and patient counseling in pituitary surgery. This study aimed to develop a clinical score to predict gross-total resection (GTR) in non-functioning pituitary adenomas (NFPAs) using externally validated machine-learning (ML) models. Methods: Clinical and radiological data were collected from two tertiary medical centers. Patients had pre- and postoperative structural T1-weighted MRI with gadolinium and T2-weighted preoperative scans. Three ML classifiers were trained on the National Hospital for Neurology and Neurosurgery dataset and tested on the Foundation IRCCS Ca’ Granda Polyclinic of Milan dataset. Feature importance analyses and hierarchical-tree inspection identified predictors of surgical complexity, which were used to create the grading score. The prognostic performance of the proposed score was compared to that of the state-of-the art TRANSSPHER grade in the external dataset. Surgical morbidity was also analyzed. Results: All ML models accurately predicted GTR, with the random forest classifier achieving the best performance (weighted-F1 score of 0.87; CIs: 0.71, 0.97). Key predictors—Knosp grade, tumor maximum diameter, consistency, and supra-sellar nodular extension—were included in the modified (m)-TRANSSPHER grade. The ROC analysis showed superior performance of the m-TRANSSPHER grade over the TRANSSPHER grade for predicting GTR in NFPAs (AUC 0.85 vs. 0.79). Conclusions: This international multi-center study used validated ML algorithms to refine predictors of surgical complexity in NFPAs, yielding the m-TRANSSPHER grade, which demonstrated enhanced prognostic accuracy for surgical complexity prediction compared to existing scales

Reappraising prediction of surgical complexity of non-functioning pituitary adenomas after transsphenoidal surgery: the modified TRANSSPHER grade

Purpose Prognostication of surgical complexity is crucial for optimizing decision-making and patient counseling in pituitary surgery. This study aimed to develop a clinical score to predict gross-total resection (GTR) in non-functioning pituitary adenomas (NFPAs) using externally validated machine-learning (ML) models. Methods Clinical and radiological data were collected from two tertiary medical centers. Patients had pre- and postoperative structural T1-weighted MRI with gadolinium and T2-weighted preoperative scans. Three ML classifiers were trained on the National Hospital for Neurology and Neurosurgery dataset and tested on the Foundation IRCCS Ca’ Granda Polyclinic of Milan dataset. Feature importance analyses and hierarchical-tree inspection identified predictors of surgical complexity, which were used to create the grading score. The prognostic performance of the proposed score was compared to that of the state-of-the art TRANSSPHER grade in the external dataset. Surgical morbidity was also analyzed. Results All ML models accurately predicted GTR, with the random forest classifier achieving the best performance (weighted-F1 score of 0.87; CIs: 0.71, 0.97). Key predictors—Knosp grade, tumor maximum diameter, consistency, and supra-sellar nodular extension—were included in the modified (m)-TRANSSPHER grade. The ROC analysis showed superior performance of the m-TRANSSPHER grade over the TRANSSPHER grade for predicting GTR in NFPAs (AUC 0.85 vs. 0.79). Conclusions This international multi-center study used validated ML algorithms to refine predictors of surgical complexity in NFPAs, yielding the m-TRANSSPHER grade, which demonstrated enhanced prognostic accuracy for surgical complexity prediction compared to existing scales

A MSFD complementary approach for the assessment of pressures, knowledge and data gaps in Southern European Seas : the PERSEUS experience

PERSEUS project aims to identify the most relevant pressures exerted on the ecosystems of the Southern European Seas (SES), highlighting knowledge and data gaps that endanger the achievement of SES Good Environmental Status (GES) as mandated by the Marine Strategy Framework Directive (MSFD). A complementary approach has been adopted, by a meta-analysis of existing literature on pressure/impact/knowledge gaps summarized in tables related to the MSFD descriptors, discriminating open waters from coastal areas. A comparative assessment of the Initial Assessments (IAs) for five SES countries has been also independently performed. The comparison between meta-analysis results and IAs shows similarities for coastal areas only. Major knowledge gaps have been detected for the biodiversity, marine food web, marine litter and underwater noise descriptors. The meta-analysis also allowed the identification of additional research themes targeting research topics that are requested to the achievement of GES. 2015 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY license.peer-reviewe