Marked efficacy of Rituximab in multifocal motor neuropathy associated with chronic lymphocytic leukemia

The authors describe a patient who presented a multifocal motor neuropathy (MMN) associated with a high anti-ganglioside antibody (anti-GM1 and anti-GD1) titer at the clinical onset of a B-cell chronic lymphocytic leukemia (B-CLL). Immunomodulation (IVIg plus cyclosporine) resulted in a neurological improvement and reduced anti-ganglioside antibody titers, both of which remained stable for at least six years. After this period, the patient had a severe relapse of the neuropathy, which was independent of the clinical course of the B-CLL. Both IVIg and cyclophosphamide were ineffective, and the patient became tetraplegic within six months; in the meantime, the patient displayed an increased antiganglioside antibody titer. Treatment with rituximab (RTX), which is designed to selectively inhibit B cell function, resulted in a dramatic, prompt and long-lasting neurological improvement as well as a reduced anti-ganglioside antibody titer. Although there are no previous reports of MMN in patients with B-CLL, the efficacy of RTX in the treatment of MMN in this patient may be considered remarkable. The expansion of B-cell clones may be a prerequisite for RTX effectiveness in MMN, and in dysimmune neuropathies in general

Marked efficacy of rituximab in multifocal motor neuropathy associated with chronic lymphocytic leukemia

The authors describe a patient who presented a multifocal motor neuropathy (MMN) associated with a high anti-ganglioside antibody (anti-GM1 and anti-GD1) titer at the clinical onset of a B-cell chronic lymphocytic leukemia (B-CLL). Immunomodulation (IVIg plus cyclosporine) resulted in a neurological improvement and reduced anti-ganglioside antibody titers, both of which remained stable for at least six years. After this period, the patient had a severe relapse of the neuropathy, which was independent of the clinical course of the B-CLL. Both IVIg and cyclophosphamide were ineffective, and the patient became tetraplegic within six months; in the meantime, the patient displayed an increased antiganglioside antibody titer. Treatment with rituximab (RTX), which is designed to selectively inhibit B cell function, resulted in a dramatic, prompt and long-lasting neurological improvement as well as a reduced antiganglioside antibody titer. Although there are no previous reports of MMN in patients with B-CLL, the eficacy of RTX in the treatment of MMN in this patient may be considered remarkable. The expansion of B-cell clones may be a prerequisite for RTX effectiveness in MMN, and in dysimmune neuropathies in general

A Simulation Model for a Hybrid-Electric Craft in Restricted Waters

Sustainable mobility is one of the most challenging issues for passenger transport inside environmental protected areas and ecologically fragile environments. To reduce the pollutant emissions, the adoption of electric or hybrid-electric solutions for crafts propulsion is a suitable option for green navigation. However, the operation in restricted basin leads also to specific critical issues for the vessel sailing, as dealing with shallow or restricted waters and transit under low air-gap bridges. The combination of these constraints with the adoption of a hybrid-electric propulsion system increases the design difficulties also for a small craft, requiring the use of advanced simulation models to assess the vessel performances. This work presents a simulation model for a small passenger craft that will operate in the Grado Lagoon. The model combines the hydrodynamic issues of manoeuvring and propulsion in restricted water with the simulation of the electric loads and capacity of the energy storage system installed onboard. The simulations performed with the developed simulation system are in accordance with data measured during trials on a prototype of the vessel. The developed model is a powerful tool for designers in order to rapidly assess the green capabilities of new projects since the early design stages

Landslide distribution and size in response to Quaternary fault activity: the Peloritani Range, NE Sicily, Italy

Landslides contribute to dismantle active mountain ranges and faults control the location of landslides. Yet, evidence of the long-term, regional dependency of landslides on active faults is limited. Previous studies focused on the transient effects of earthquakes on slope stability in compressive and transcurrent regimes. Here we show that in the Peloritani range, NE Sicily, Italy, one of the fastest uplifting areas in the Mediterranean, a clear geographical association exists between large bedrock landslides and active normal faults of the Messina Straits graben. By interpreting aerial photographs, we mapped 1590 landslides and sackungs and 626 fault elements and their facets in a 300 km2 area in the eastern part of the range. We used the new landslide and fault information, in combination with prior geological and seismic information, to investigate the association between bedrock landslides and faults. We find that the distribution and abundance of landslides is related to the presence of large active normal faults, and matches the pattern of the local historical seismicity. Landslide material is more abundant along the East Peloritani Fault System where the long-term activity of the faults, measured by the average yearly geological moment rate, is larger than in the West Peloritani Fault System where landslides are less abundant. Along the fault systems landslide material concentrates where the cumulated fault throws are largest. We conclude that large landslides and their cumulated volume are sensitive to local rates of tectonic deformation, and discriminate the deformation of the single fault segments that dissect the Peloritani range. Our findings are a direct test of landscape evolution models that predict higher rates of landslide activity near active faults. Our work opens up the possibility of exploiting accurate landslide and fault maps, in combination with geological and seismic information, to characterize the long-term seismic history of poorly instrumented active regions. Copyright © 2015 The Authors Earth Surface Processes and Landforms Published by John Wiley & Sons Lt

Hybrid-electric solutions for the propulsion of a luxury sailing yacht

The application of hybrid-electric solutions for the propulsion of pleasure crafts is increasing in recent years. The more restrictive regulations on pollutants and the growing sensibility of the people on greenness make this solution even more attractive. In case the craft is stationing in a marine equipped with renewable energy sources, the attractiveness of such a solution increases even more. Besides, a hybrid-electric configuration can reduce the consumptions and, consequently, the operative costs against an initial higher investment. Therefore, hybrid-electric propulsion could give a benefit to raise a stationary market, as the pleasure craft one. This study compares in terms of consumptions three different solutions, besides conventional diesel one, for the propulsion of a sailing yacht

photo geology of the montefalco quaternary basin umbria central italy

ABSTRACTWe present a photo-geological map for the 185 km2 fault-bounded, Montefalco Basin, Umbria, Central Italy. The basin formed in the Quaternary in response to extensional tectonics dissecting folds and thrusts of the northern Apennines range. To prepare the 1:25,000 geological map, we integrated geological and morphological information obtained through the visual analysis of three sets of aerial photographs of different age, the collection of new field data, and the review of pre-existing surface and sub-surface geological data. We show that systematic interpretation of aerial photographs contributed to improving the geological mapping, providing information not readily available through traditional field mapping. We expect that the new map will be used for different types of geological and geomorphological investigations, including studies of active tectonic, Quaternary morpho-tectonics, sedimentological and stratigraphic analyses, mining and exploration investigations, and the analysis of landslide..

Communicating Seismic Risk Information: The Effect of Risk Comparisons on Risk Perception Sensitivity

Communicating seismic risk to individuals can be difficult for an institution because it involves providing technical and scientific information, including the low probability of an adverse event, that is not always easy to understand. One way to facilitate understanding of low probabilities is to provide comparisons with the probability of occurrence of other more familiar events. In a randomized trials experiment, we investigated the effect of providing individuals with a set of risk comparisons on their sensitivity to different levels of seismic risk (1 in 100, 1 in 1,000, and 1 in 10,000). The findings show that providing risk comparisons increased individual risk sensitivity to information about the likelihood of experiencing a seismic event. Our findings are explained by the evaluability hypothesis, which states that a single probability value is better understood if the recipient is given some reference data to evaluate it. Our results have implications for disaster risk communication, providing ways to increase risk awareness and, consequently, disaster prevention

Civil Protection Achievements and Critical Issues in Seismology and Earthquake Engineering Research

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COVID-19 infection: the perspectives on immune responses.

Funder: National Key R&D Program of China (2018YFA0107500), Suzhou 2020 Emergency Innovation Funding on COVID-19 Infection, the National Natural Science Foundation of China (81530043, 81861138015, 31771641 and 81571612)Funder: Italian Ministry of Healt

Serological Response to BNT162b2 Anti-SARS-CoV-2 Vaccination in Patients with Inflammatory Rheumatic Diseases: Results From the RHEUVAX Cohort

Objective: In the light of the current COVID-19 epidemic and the availability of effective vaccines, this study aims to identify factors associated with non-response to anti-SARS-CoV-2 vaccines as immunological alteration associated with immune rheumatic diseases (IRD) and immunosuppressive medications may impair the response to vaccination. Methods: Volunteers in the health profession community with IRD, age, and sex-matched controls (CTRL) who underwent vaccination with two doses of BNT162b2 were recruited for this study. Anti-Trimeric Spike protein antibodies were assayed eight ± one weeks after the second vaccine dose. Univariate and logistic regression analyses were performed to identify factors independently associated with non-response and low antibody titers. Results: Samples were obtained from 237 IRD patients (m/f 73/164, mean age 57, CI 95% [56-59]): 4 autoinflammatory diseases (AI), 62 connective tissue diseases (CTD), 86 rheumatoid arthritis (RA), 71 spondylarthritis (SpA) and 14 vasculitis (Vsc). 232 CTRL were recruited (m/f 71/161, mean age 57, CI 95% [56-58]). Globally, IRD had a lower seroconversion rate (88.6% vs 99.6%, CI 95% OR [1.61-5.73], p<0.001) and lower antibody titer compared to controls (median (IQR) 403 (131.5-1012) versus 1160 (702.5-1675), p<0.001). After logistic regression, age, corticosteroid (CCS), Abatacept and Mycophenolate Mofetil (MMF) use were associated with non-response. Lower antibody titer was associated with the use of MMF, ABA, CCS, Rituximab, tumor necrosis factor inhibitor, JAK inhibitors, and higher age. Conclusion: The response to anti-SARS-CoV-2 vaccines is often impaired in IRD patients under treatment and may pose them at higher risk of severe COVID-19. Specific vaccination protocols are desirable for these patients