Odontogenic keratocyst and uterus bicornis in nevoid basal cell carcinoma syndrome: Case report and literature review

Nevoid basal cell carcinoma syndrome (NBCCS), an autosomal dominant disorder with a high degree of penetrance and variable expressivity, is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. More than 100 minor criteria have been described but two major and one minor criteria or one major and three minor criteria are necessary for the diagnosis. In this report we present a 8 year old girl affected by NBCCS showing an uterus bicornis, an hitherto unreported association. However further researches are needed to confirm the association between NBCCS and mullerian fusion defects and to assess the hypothesis that focuses on chromosome 9 the mutant gene for NBCCS and fusion defects of female genital tract

Metric-like Lagrangian Formulations for Higher-Spin Fields of Mixed Symmetry

We review the structure of local Lagrangians and field equations for free bosonic and fermionic gauge fields of mixed symmetry in flat space. These are first presented in a constrained setting extending the metric formulation of linearized gravity, and then the ($\gamma$-)trace constraints on fields and gauge parameters are eliminated via the introduction of auxiliary fields. We also display the emergence of Weyl-like symmetries in particular classes of models in low space-time dimensions.Comment: 136 pages, LaTeX. References added. Final version to appear in La Rivista del Nuovo Cimento

A study of gas contaminants and interaction with materials in RPC closed loop systems

Resistive Plate Counters (RPC) detectors at the Large Hadron Collider (LHC) experiments use gas recirculation systems to cope with large gas mixture volumes and costs. In this paper a long-term systematic study about gas purifiers, gas contaminants and detector performance is discussed. The study aims at measuring the lifetime of purifiers with unused and used cartridge material along with contaminants release in the gas system. During the data-taking the response of several RPC double-gap detectors was monitored in order to characterize the correlation between dark currents, filter status and gas contaminants

Status of the production of GEM chambers for the CMS experiment at Large Hadron Collider

The High Luminosity LHC phase includes an upgrade to the muon stations for the CMS Experiment. CMS trigger and muon identification performance will be crucial, and it is, therefore, necessary to install new GEM stations to extend acceptance in the high-{\eta} region. An explanation of the quality control test and an update on the status of production will be provided

Wind direction data from a coastal HF radar system in the gulf of naples (central mediterranean sea)

Results on the accuracy of SeaSonde High Frequency (HF) radar wind direction measurements in the Gulf of Naples (Southern Tyrrhenian Sea, Central Mediterranean Sea) are here presented. The investigation was carried out for a winter period (2 February-6 March) and for one summer month (August) of the reference year 2009. HF radar measurements were compared with in situ recordings from a weather station and with model data, with the aim of resolving both small scale and large scale dynamics. The analysis of the overall performance of the HF radar system in the Gulf of Naples shows that the data are reliable when the wind speed exceeds a 5 m/s threshold. Despite such a limitation, this study confirms the potentialities of these systems as monitoring platforms in coastal areas and suggests further efforts towards their improvement

An analysis of materials used in the RPC detector and in the closed loop gas system of CMS at the LHC.

The results are reported of the study of materials used in the CERN Closed Loop recirculation gas system currently under test with the RPC muon detectors in the CMS experiment at the LHC. Studies include a sampling campaign in a low-radiation environment (cosmic rays at the CERN ISR test site). We describe the dedicated RPC chamber tests, the chemical analysis of the filters and gas used, and discuss the results of the Closed Loop system

Safety and efficacy evaluation in vivo of a cationic nucleolipid nanosystem for the nanodelivery of a ruthenium(Iii) complex with superior anticancer bioactivity

Selectivity and efficacy towards target cancer cells, as well as biocompatibility, are current challenges of advanced chemotherapy powering the discovery of unconventional metal‐based drugs and the search for novel therapeutic approaches. Among second‐generation metal‐based chemotherapeutics, ruthenium complexes have demonstrated promising anticancer activity cou-pled to minimal toxicity profiles and peculiar biochemical features. In this context, our research group has recently focused on a bioactive Ru(III) complex—named AziRu—incorporated into a suite of ad hoc designed nucleolipid nanosystems to ensure its chemical stability and delivery. In-deed, we proved that the structure and properties of decorated nucleolipids can have a major impact on the anticancer activity of the ruthenium core. Moving in this direction, here we describe a preclinical study performed by a mouse xenograft model of human breast cancer to establish safety and efficacy in vivo of a cationic Ru(III)‐based nucleolipid formulation, named HoThyRu/DOTAP, endowed with superior antiproliferative activity. The results show a remarkable reduction in tu-mour with no evidence of animal suffering. Blood diagnostics, as well as biochemical analysis in both acute and chronic treated animal groups, demonstrate a good tolerability profile at the therapeutic regimen, with 100% of mice survival and no indication of toxicity. In addition, ruthenium plasma concentration analysis and tissue bioaccumulation were determined via appropriate sam-pling and ICP‐MS analysis. Overall, this study supports both the efficacy of our Ru‐containing nanosystem versus a human breast cancer model and its safety in vivo through well‐tolerated animal biological responses, envisaging a possible forthcoming use in clinical trials

A 1 m3^3 Gas Time Projection Chamber with Optical Readout for Directional Dark Matter Searches: the CYGNO Experiment

The aim of the CYGNO project is the construction and operation of a 1~m$^3$ gas TPC for directional dark matter searches and coherent neutrino scattering measurements, as a prototype toward the 100-1000~m$^3$ (0.15-1.5 tons) CYGNUS network of underground experiments. In such a TPC, electrons produced by dark-matter- or neutrino-induced nuclear recoils will drift toward and will be multiplied by a three-layer GEM structure, and the light produced in the avalanche processes will be readout by a sCMOS camera, providing a 2D image of the event with a resolution of a few hundred micrometers. Photomultipliers will also provide a simultaneous fast readout of the time profile of the light production, giving information about the third coordinate and hence allowing a 3D reconstruction of the event, from which the direction of the nuclear recoil and consequently the direction of the incoming particle can be inferred. Such a detailed reconstruction of the event topology will also allow a pure and efficient signal to background discrimination. These two features are the key to reach and overcome the solar neutrino background that will ultimately limit non-directional dark matter searches.Comment: 5 page, 7 figures, contribution to the Conference Records of 2018 IEEE NSS/MI

Gas Analysis and Monitoring Systems for the RPC Detector of CMS at LHC

The Resistive Plate Chambers (RPC) detector of the CMS experiment at the LHC proton collider (CERN, Switzerland) will employ an online gas analysis and monitoring system of the freon-based gas mixture used. We give an overview of the CMS RPC gas system, describe the project parameters and first results on gas-chromatograph analysis. Finally, we report on preliminary results for a set of monitor RPC.Comment: 9 pages, 8 figures. Presented by Stefano Bianco (Laboratori Nazionali di Frascati dell'INFN) at the IEEE NSS, San Diego (USA), October 200