A scoring system for biliary atresia: Is this the right one?

A clinical diagnostic score for biliary atresia in discusse

La Colangiopatia autoimmune in età evolutiva: caratteristiche cliniche, istopatologiche e storia naturale

Le malattie autoimmuni del fegato nell’adulto comprendono tre entità maggiori tra cui, una, l’epatite autoimune, caratterizzata da danno prevalentemente epatocitario, e due, la Cirrosi Biliare Primitiva e la Colangite Sclerosante Primitiva, contraddistinte da lesioni immunomediate prevalentemente a carico delle vie biliari. Tale attuale classificazione risulta insufficiente; da molti anni esiste un fitto dibattito intorno ad una entità attigua alla Epatite Autoimmune, denominata Colangite Autoimmune caratterizzata da lesioni istologiche dei dotti biliari e profilo anticorpale tipico della Epatite Autoimmune tipo 1. Nella patologia pediatrica, caratterizzata in generale da una maggiore attività infiammatoria e dall’assenza della Cirrosi Biliare Primitiva, l’opportunità di considerare la Colangite Autoimmune come entità autonoma è stata occasionalmente suggerita. Ad oggi, tuttavia, non esistono in letteratura casistiche omogenee pediatriche. Questa tesi è il risultato di uno studio multicentrico che ha interessato la Clinica Pediatrica di Pisa, l’IRCSS Burlo Garofalo di Trieste e il Service d’Hépatologie Pédiatrique de l’Höpital di Bicētre. Sono stati studiati complessivamente 55 pazienti affetti da Colangite Autoimmune le cui caratteristiche cliniche, istopatologiche, radiologiche ed evolutive sono state registrate. Dallo studio di tale casistiche emerge l’immagine di una malattia caratterizzata da colestasi bioumorale con gradi variabili di citolisi, danno istologico delle vie biliari di tutte le dimensioni talora evidenziabile all’imaging delle vie biliari, malattia infiammatoria aspecifica del tubo digerente associata nel 90% dei casi. Il Follow-up di tale malattia restituisce l’immagine di una storia naturale sensibile alla terapia immunosoppressiva, in maniera simile all’epatite autoimmune, la cui evoluzione può essere bruscamente cambiata dall’emergere delle complicazioni tipiche della Colangite Sclerosante, stadio finale evolutivo della Colangite Autoimmune, e richiedere infine un trapianto epatico

Global profiling of viral and cellular non-coding RNAs in Epstein-Barr virus-induced lymphoblastoid cell lines and released exosome cargos.

Abstract The human EBV-transformed lymphoblastoid cell line (LCL), obtained by infecting peripheral blood monocular cells with Epstein–Barr Virus, has been extensively used for human genetic, pharmacogenomic, and immunologic studies. Recently, the role of exosomes has also been indicated as crucial in the crosstalk between EBV and the host microenvironment. Because the role that the LCL and LCL exosomal cargo might play in maintaining persistent infection, and since little is known regarding the non-coding RNAs of LCL, the aim of our work was the comprehensive characterization of this class of RNA, cellular and viral miRNAs, and cellular lncRNAs, in LCL compared with PBMC derived from the same donors. In this study, we have demonstrated, for the first time, that all the viral miRNAs expressed by LCL are also packaged in the exosomes, and we found that two miRNAs, ebv-miR-BART3 and ebv-miR-BHRF1-1, are more abundant in the exosomes, suggesting a microvescicular viral microRNA transfer. In addition, lncRNA profiling revealed that LCLs were enriched in lncRNA H19 and H19 antisense, and released these through exosomes, suggesting a leading role in the regulation of the tumor microenvironment

Large active-area superconducting microwire detector array with single-photon sensitivity in the near-infrared

Superconducting nanowire single photon detectors (SNSPDs) are the highest-performing technology for time-resolved single-photon counting from the UV to the near-infrared. The recent discovery of single-photon sensitivity in micrometer-scale superconducting wires is a promising pathway to explore for large active area devices with application to dark matter searches and fundamental physics experiments. We present 8-pixel $1 mm^2$ superconducting microwire single photon detectors (SMSPDs) with $1\,\mathrm{\mu m}$-wide wires fabricated from WSi and MoSi films of various stoichiometries using electron-beam and optical lithography. Devices made from all materials and fabrication techniques show saturated internal detection efficiency at 1064 nm in at least one pixel, and the best performing device made from silicon-rich WSi shows single-photon sensitivity in all 8 pixels and saturated internal detection efficiency in 6/8 pixels. This detector is the largest reported active-area SMSPD or SNSPD with near-IR sensitivity published to date, and the first report of an SMSPD array. By further optimizing the photolithography techniques presented in this work, a viable pathway exists to realize larger devices with $cm^2$-scale active area and beyond

Readout technologies for directional WIMP Dark Matter detection

The measurement of the direction of WIMP-induced nuclear recoils is a compelling but technologically challenging strategy to provide an unambiguous signature of the detection of Galactic dark matter. Most directional detectors aim to reconstruct the dark-matter-induced nuclear recoil tracks, either in gas or solid targets. The main challenge with directional detection is the need for high spatial resolution over large volumes, which puts strong requirements on the readout technologies. In this paper we review the various detector readout technologies used by directional detectors. In particular, we summarize the challenges, advantages and drawbacks of each approach, and discuss future prospects for these technologies

Search for dark matter produced in association with bottom or top quarks in √s = 13 TeV pp collisions with the ATLAS detector

A search for weakly interacting massive particle dark matter produced in association with bottom or top quarks is presented. Final states containing third-generation quarks and miss- ing transverse momentum are considered. The analysis uses 36.1 fb−1 of proton–proton collision data recorded by the ATLAS experiment at √s = 13 TeV in 2015 and 2016. No significant excess of events above the estimated backgrounds is observed. The results are in- terpreted in the framework of simplified models of spin-0 dark-matter mediators. For colour- neutral spin-0 mediators produced in association with top quarks and decaying into a pair of dark-matter particles, mediator masses below 50 GeV are excluded assuming a dark-matter candidate mass of 1 GeV and unitary couplings. For scalar and pseudoscalar mediators produced in association with bottom quarks, the search sets limits on the production cross- section of 300 times the predicted rate for mediators with masses between 10 and 50 GeV and assuming a dark-matter mass of 1 GeV and unitary coupling. Constraints on colour- charged scalar simplified models are also presented. Assuming a dark-matter particle mass of 35 GeV, mediator particles with mass below 1.1 TeV are excluded for couplings yielding a dark-matter relic density consistent with measurements