Myotonic dystrophy type 1 and high ventricular vulnerability at the electrophysiological evaluation: ICD yes or not?

A significant number of sudden death (SD) is observed in myotonic dystrophy (DM1) despite pacemaker implantation and some consider the ICD to be the preferential device in patients with conduction disease. According to the latest guidelines, prophylactic ICD implantation in patients with neuromuscular disorder should follow the same recommendations of non-ischemic dilated cardiomyopathy, being reasonable when pacing is needed. We here report a case of DM1 patient who underwent ICD implantation even in the absence of conduction disturbances on ECG and ventricular dysfunction/fibrosis at cardiac magnetic resonance. The occurrence of syncope, non-sustained ventricular tachycardias at 24-Holter ECG monitoring and a family history of SD resulted associated with ventricular fibrillation inducibility at electrophysiological study, favouring ICD implantation. On our advice, DM1 patient with this association of SD risk factors should be targeted for ICD implantation

Exploring drivers of overnight stays and same-day visits in the tourism sector

: We employ mobile network data referred to the area of Lombardy in Italy to investigate alternative touristic behaviours, such as same-day visits and overnight stays in Italy. We show that larger availability of tourism accommodations, cultural and natural endowments are relevant factors explaining overnight stays. Conversely, temporary entertainment and transportation facilities increase municipalities attractiveness for same-day visits. The results also highlight a trade-off in the capability of municipalities of being attractive in connection to both the tourism behaviours. For instance, higher tourists arrivals are observed in areas receiving limited visitors, coming from municipalities with low same-day visits outflows. We highlight mobile data offer an adequate level of spatial and temporal granularity and can be thus employed to support policy makers in the design of effective tourist management strategies

Geochronology of late Variscan magmatism of Sardinia: a review

Sardinia represents a southern transect of the Variscan Belt and is classically divided from SW to NE into a fold and thrust belt Foreland, Nappe zone and Axial zone. This latter high-grade domain is separated from the Nappe zone by the so-called Posada-Asinara Line. The whole metamorphic basement is intruded by many calc-alkaline coalescent plutons forming the Corsica-Sardinia Batholith. The timing of magmatism, in Sardinia, is broadly referable to a large interval in the range of 320-280 Ma. Recent geological maps coupled to several chronological systematics, point out to recognize two main post-collisional magmatic peaks clustered at about 305 Ma (Older Magmatic Peak, OMP) and at 285 Ma (Young Magmatic Peak, YMP), respectively. Plutons intruding different parts of the Sardinian basement show different geological styles. Among the OMP, main differences regard: (a) the granodiorite/granite volume ratio in the main plutons; (b) the abundance of peraluminous rock-types; (c) the occurrence of mafic intrusions; (d) the abundance of late-magmatic dyke swarms. The Axial zone is dominated by monzogranites and subordinate granodiorites and leucogranites (320-307 Ma); tonalites and granodiorites (305-300 Ma), along with peraluminous granites, characterize the inner Nappe zone (i.e., Goceano, Baronie, Barbagia). Remarkably, the oldest intrusions (i.e., Barrabisa and Santa Maria: 320 Ma) are foliated and may represent a prebatholith magmatic phase. Plutons occurring in the external Nappe zone and the Foreland are generally dominated by granodiorites (e.g., Arbus, Ogliastra, Sàrrabus: 305-300 Ma) associated to small gabbronoritic bodies. The YMP is marked in the Axial zone by large leucogranite intrusions (Monte Lerno); gabbroic intrusions are present at 285-280 Ma. The YMP is dominant in the external Nappe zone and in the Foreland. This peak include monzogranites and leucogranites with minor granodiorites; specific characters are: (a) common occurrence of F-bearing, ferroan, ilmeniteseries granitoids; (b) slightly peraluminous character; (c) very shallow emplacement levels, with common greisen alteration; (d) presence of Sn-W-Mo and F ores; (e) association to swarms of tholeiitic mafic dikes. The timing and distribution of Sardinian intrusive magmatism are tentatively framed during the post-collisional evolution of the chain, in response to progressive lithospheric delamination along a N-S direction. In this model, the passive upwelling of hot astenosphere triggered dehydration melting at lower to intermediate crustal levels, associated with minor melting of the lithospheric mantle. Several major issues emerge from this schematic picture, including (a) the precise timing of the magmatic peaks, (b) the significance of the gap between them, (c) the difference in volumes and spatial distribution of the main rock-suites, (d) the geological and petrological frame at the district- to single pluton-, up to regional scale, and (e) the age of mafic dyke swarms

Evaluating the impact of transport mechanisms on web performance for effective web access

Peer reviewedPostprin

Data-Driven Methodology for Knowledge Graph Generation Within the Tourism Domain

The tourism and hospitality sectors have become increasingly important in the last few years and the companies operating in this field are constantly challenged with providing new innovative services. At the same time, (big-) data has become the 'new oil' of this century and Knowledge Graphs are emerging as the most natural way to collect, refine, and structure this heterogeneous information. In this paper, we present a methodology for semi-automatic generating a Tourism Knowledge Graph (TKG), which can be used for supporting a variety of intelligent services in this space, and a new ontology for modelling this domain, the Tourism Analytics Ontology (TAO). Our approach processes and integrates data from Booking.com, Airbnb, DBpedia, and GeoNames. Due to its modular structure, it can be easily extended to include new data sources or to apply new enrichment and refinement functions. We report a comprehensive evaluation of the functional, logical, and structural dimensions of TKG and TAO

Resíduo do processo de fabricação de papel reciclado - potencial de uso como insumo agrícola e/ou florestal.

Resumo

Promising use of filgotinib in patients affected by systemic sclerosis: Preliminary data from a case series of 5 patients

Objective: To assess the efficacy and safety of Filgotinib for the management of cutaneous, visceral and articular involvement in pat ients affected by systemic sclerosis. Methods: 5 patients affected by SSc referring to the Scleroderma Units of Modena and Reggio Emilia between October 2021 and February 2023, were enrolled. Patients received 200 mg of Filgotinib once daily for a period of 52 weeks. Skin, articular and visceral organ involvement were evaluated at the baseline and every 12 weeks after the start of the treatment. Results: A significant improvement of articular involvement was seen at 12th week. All patients confirmed a significant amelioration in articular involvement at 52th week, with a significant reduction in TJ, SDAI/CDAI, DAS28-CRP. PDUS performed after 24 and 52 weeks of treatment supported the remission. A significant decrease of mRSS, improvement of other skin manifestations and a stabilization of ILD, assessed through HRCT and PFTs, were noticed in patients with dcSSc. The 2 patients with dsSSC showed a clinical improvement assessed using the CRISS score. No drug-related side effects were recorded and neither patients discontinued the treatment. No deaths were reported. Conclusion: Filgotinib was safe, effective and well tolerated in the treatment of articular and visceral involvement in patients affected by SSc