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Abstract Aims Carpal tunnel (CT) syndrome is a recognized red‐flag of cardiac amyloidosis (CA) and increased cardiovascular (CV) morbidity. We designed this study to characterize the CV profile of patients with CT syndrome at the time of first surgery and to identify high‐risk presentations. Methods and results We retrospectively reviewed 643 patients who underwent CT surgery between 2007 and 2019. Of them, 130 patients (77 years, 45% male patients, left ventricular ejection fraction 62%) with available CV characterization within ±12 months from CT surgery were included. Abnormal loading conditions causing cardiac left ventricular hypertrophy (LVH) were investigated to distinguish explained LVH (Ex‐LVH) from unexplained LVH (Un‐LVH). LVH was found in 66 (51%) patients, 33% of them presented Un‐LVH. Compared with the others, Un‐LVH patients were older (77 and 75 vs. 70 years in Un‐LVH, Ex‐LVH, and non‐LVH, respectively; P = 0.002), had higher rates of electrocardiogram‐echo discrepancy (70%, 14.3%, and 1.6%, respectively; P

The Habitat Directive of European Union lists Costal Lagoons (habitat code 1150*) among priority habitats because they are in danger of disappearance. Natural ecosystems may recover from anthropogenic perturbations; however, the recovery can follow natural restoration or it can be redirected through ecological restoration by anthropogenic intervention. Accordingly, by collecting the available theoretical indications for restoration of estuarine and coastal areas, a methodological approach was detailed andit can be summarised into five issues: (i) Environmental context from which it began; (ii) Desired state to be achieved; (iii) Policies and socio-economic context; (iv) Typology of recovery and/or improvement of habitats and ecosystems; and (v) Methods for monitoring the impact of the project. The project strategy, management and measures of LIFE Lagoon Refresh were also presented and discussed, as a case study for the implementation of the multidisciplinary approach for restoration ecology in transitional waters. The project takes place in the northern Venice Lagoon (Italy), started in 2017 and it lasts 5 years. In the Venice Lagoon, since the 20th century, strong reductions of the typical salinity gradient of buffer areas between lagoon and mainland, and of reedbed extensions have occurred due to historic human interventions, with negative consequences on coastal lagoon habitats. To improve the conservation status of habitats and biodiversity of the area, the LIFE Lagoon Refresh project included several conservative actions, which are (i) the diversion of a freshwater flow from the Sile River into the lagoon; (ii) the restoration of intertidal morphology, through biodegradable structures; (iii) the reed and aquatic angiosperm transplantations with the involvement of local fishermen and hunters, and (iv) the reduction of hunting and fishing pressures in the intervention area. To achieve the restoration of the lagoon environment, the strategy of the project covered a combination of different aspects and tools, such as planning activities, through the involvement of local Institutions and communities; stakeholder’s involvement to increase awareness of environment conservation and socioeconomic value improvement; an ecological engineering approach; numerical models as supporting tool for planning and managing of conservation actions; environmental monitoring performed before and after the conservation actions.

ObjectiveThe aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.MethodsThis Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management. The Registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, thus enhancing international collaboration and data sharing for research purposes. The Registry is practical enough to be easily modified to meet future needs regarding VEXAS syndrome.ResultsTo date (April 22nd, 2022), 113 Centers from 23 Countries in 4 continents have been involved; 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) are currently able to access the registry for data entry (or data sharing) and collection. The Registry includes 4,952 fields organized into 18 instruments designed to fully describe patient's details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access.ConclusionThis international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease, with the final goal to obtain real-world evidence for daily clinical practice, especially in relation to the comprehension of this disease about the natural history and the possible therapeutic approaches. This Project can be found on https://clinicaltrials.gov NCT05200715.

ObjectiveAim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder.MethodsThis Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still's disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions.ResultsStarting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access.ConclusionsThis international Registry for patients with Still's disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of this Registry, which has been implemented to significantly improve the overall management of patients with Still's disease. NCT 05200715 available at https://clinicaltrials.gov/.

Abstract Background Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion. Methods A panel of 10 experts (9 rheumatologists and 1 pediatrician) was established. The first step was dedicated to a comprehensive literature review and development of statements. Two separate literature searches were performed on the MEDLINE (Pubmed), EMBASE, and BIOSIS databases through April 2018 to identify (1) differences and similarities between AOSD and pediatric Still’s disease (systemic juvenile idiopathic arthritis [SJIA]) and (2) the efficacy and safety of IL-1 inhibitors in AOSD treatment. In the second step, the statements were submitted in a Delphi process to a panel of 67 rheumatologists. Consensus threshold was set at 66%: positive, > 66% of voters selected scores 3 to 5; negative, > 66% of voters selected scores 1 or 2. In the third step, the voting results were analyzed, and the statements were finalized. Results Eleven statements were developed. Forty-six of 67 rheumatologists (72%) participated in the Delphi process. A positive consensus was reached after the first round of voting and was full (> 95%) on the majority of statements. A large consensus was achieved in considering AOSD and SJIA as the same disease. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. Because of the lack of head-to-head comparisons, a different profile of efficacy among IL-1 inhibitors could not be established. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. The lack of studies comparing early versus late treatment did not allow to draw conclusions; however, data from SJIA suggest a better response in early treatment. Conclusions The Delphi method was used to develop recommendations that we hope will help clinicians in the management of patients with AOSD refractory to conventional therapies.

Oxidized polyvinyl alcohol (OxPVA) is a new polymer for the fabrication of nerve conduits (NCs). Looking for OxPVA device optimization and coupling it with a natural sheath may boost bioactivity. Thus, OxPVA/chitosan sponges (ChS) as hybrid scaffolds were investigated to predict in the vivo behaviour of two-layered NCs. To encourage interaction with cells, ChS were functionalized with the self-assembling-peptide (SAP) EAK, without/with the laminin-derived sequences -IKVAV/-YIGSR. Thus, ChS and the hybrid scaffolds were characterized for mechanical properties, ultrastructure (Scanning Electron Microscopy, SEM), bioactivity, and biocompatibility. Regarding mechanical analysis, the peptide-free ChS showed the highest values of compressive modulus and maximum stress. However, among +EAK groups, ChS+EAK showed a significantly higher maximum stress than that found for ChS+EAK-IKVAV and ChS+EAK-YIGSR. Considering ultrastructure, microporous interconnections were tighter in both the OxPVA/ChS and +EAK groups than in the others; all the scaffolds induced SH-SY5Y cells’ adhesion/proliferation, with significant differences from day 7 and a higher total cell number for OxPVA/ChS+EAK scaffolds, in accordance with SEM. The scaffolds elicited only a slight inflammation after 14 days of subcutaneous implantation in Balb/c mice, proving biocompatibility. ChS porosity, EAK 3D features and neuro-friendly attitude (shared with IKVAV/YIGSR motifs) may confer to OxPVA certain bioactivity, laying the basis for future appealing NCs.

In benthic studies, the identification of organisms at the species level is known to be the best source for ecological and biological information even if time-consuming and expensive. However, taxonomic sufficiency (TS) has been proposed as a short-cut method for quantifying changes in biological assemblages in environmental monitoring. In this paper, we set out to determine whether and how the taxonomic complexity of a benthic assemblage influences the results of TS at two different long-term ecological research (LTER) sites in the Po delta region (north-eastern Italy). Specifically, we investigated whether TS can be used to detect natural and human-driven patterns of variation in benthic assemblages from lagoonal soft bottoms. The first benthic dataset was collected from 1996 to 2015 in a “choked” lagoon, the Valli di Comacchio, a lagoon characterised by long water residence times and heavy eutrophication, while the second was collected from 2004 to 2010 in a “leaky” lagoon, the Sacca di Goro, a coastal area with human pressure limited to aquaculture. Univariate and multivariate statistical analyses were used to assess differences in the taxonomic structure of benthic assemblages and to test TS on the two different datasets. TS seemed to work from species to family level at both sites, despite a higher natural variability of environmental conditions combined with multiple anthropogenic stressors. Therefore, TS at the family level may represent effective taxonomic surrogates across a range of environmental contexts in lagoon environments. Since the structure of the community and the magnitude of changes could influence the efficiency of taxonomic surrogates and data transformations in long-term monitoring, we also suggest periodic analyses at finer taxonomic levels in order to check the efficiency of the application of taxonomic substitutes in routine monitoring programmes in lagoon systems.

The determination of the trophic status of transitional ecosystems from the physico-chemical and biological point of view is one of the requirements of the European Water Framework Directive (WFD 2000/60/EC). In Italy, its determination is implemented by the Regional Agencies for Environmental Protection (ARPAs) that have activated multi-annual monitoring programs. However, as the availability of funds is increasingly scarce, the number of environmental parameters to detect environmental changes should be conveniently managed. The high number of environmental parameters, nutrient and macrophyte datasets available for the LTER-Italia site “Venice lagoon” can be an useful tool to analyze the trophic changes over recent years and to foresee environmental evolutions. Nutrient data on a spatial basis have been available since 1948, whereas macroalgal maps date back to 1980. The aim of this paper is to highlight the changes of the trophic status of the lagoon since the middle of the 20th century by considering the concentrations of nutrients in the surface sediments and in the water column, the variation of some physico-chemical parameters and the biomass of macroalgae and also to foresee the way it will possibly evolve. In fact, after many anthropogenic impacts that in the second half of the 20th century affected the lagoon, starting from the year 2010, the ecological status is progressively improving. Nutrients show a significant reduction both in the water column and in surface sediments, and the macrophytes are represented by species of higher ecological value while the opportunistic species such as the Ulvaceae are in strong regression.

Adenosine A2A receptors (A2AR) and dopamine D2 receptors (D2R) are known to be involved in the physiological response to hypoxia, and their expression/activity may be modulated by chronic sustained or intermittent hypoxia. To date, A2AR and D2R can form transient physical receptor–receptor interactions (RRIs) giving rise to a dynamic equilibrium able to influence ligand binding and signaling, as demonstrated in different native tissues and transfected mammalian cell systems. Given the presence of A2AR and D2R in type I cells, type II cells, and afferent nerve terminals of the carotid body (CB), the aim of this work was to demonstrate here, for the first time, the existence of A2AR–D2R heterodimers by in situ proximity ligation assay (PLA). Our data by PLA analysis and tyrosine hydroxylase/S100 colocalization indicated the formation of A2AR–D2R heterodimers in type I and II cells of the CB; the presence of A2AR–D2R heterodimers also in afferent terminals is also suggested by PLA signal distribution. RRIs could play a role in CB dynamic modifications and plasticity in response to development/aging and environmental stimuli, including chronic intermittent/sustained hypoxia. Exploring other RRIs will allow for a broad comprehension of the regulative mechanisms these interactions preside over, with also possible clinical implications.

Microplastic contamination of the benthic invertebrate fauna in Terra Nova Bay (Ross Sea, Antarctica) was determined. Twelve macrobenthic species, characterized by different feeding strategies, were selected at 3 sampling sites at increasing distance from the Italian Scientific Base (Mario Zucchelli, Camp Icarus, Adelie Cove). The 83% of the analyzed macrobenthic species contained microplastics (0.01–3.29 items mg−1). The size of the particles, measured by Feret diameter, ranged from 33 to 1000 µm with the highest relative abundance between 50 and 100 µm. Filter-feeders and grazers displayed values of microplastic contamination from 3 to 5 times higher than omnivores and predators, leading to the hypothesis that there is no evident bioaccumulation through the food web. The prevalent polymers identified by micro-FTIR were nylon (86%) and polyethylene (5%); other polymers identified in Antarctic benthos were polytetrafluoroethylene, polyoxymethylene, phenolic resin, polypropylene, polystyrene resin and XT polymer. Keywords: Antarctica, Benthos, Microplastics, Food web, FTIR, Nile red