von Willebrand disease (VWD) is a bleeding disorder caused by inherited defects in the concentration, structure, or function of von Willebrand factor (VWF). VWD is classified into three primary ...categories. Type 1 includes partial quantitative deficiency, type 2 includes qualitative defects, and type 3 includes virtually complete deficiency of VWF. VWD type 2 is divided into four secondary categories. Type 2A includes variants with decreased platelet adhesion caused by selective deficiency of high-molecular-weight VWF multimers. Type 2B includes variants with increased affinity for platelet glycoprotein Ib. Type 2M includes variants with markedly defective platelet adhesion despite a relatively normal size distribution of VWF multimers. Type 2N includes variants with markedly decreased affinity for factor VIII. These six categories of VWD correlate with important clinical features and therapeutic requirements. Some VWF gene mutations, alone or in combination, have complex effects and give rise to mixed VWD phenotypes. Certain VWD types, especially type 1 and type 2A, encompass several pathophysiologic mechanisms that sometimes can be distinguished by appropriate laboratory studies. The clinical significance of this heterogeneity is under investigation, which may support further subdivision of VWD type 1 or type 2A in the future.
Wind‐formed features are abundant in Oxia Planum (Mars), the landing site of the 2022 ExoMars mission, which shows geological evidence for a past wet environment. Studies of aeolian bedforms at the ...landing site were focused on assessing the risk for rover trafficability, however their potential in recording climatic fluctuations has not been explored. Here we show that the landing site experienced multiple climatic changes in the Amazonian, which are recorded by an intriguing set of ridges that we interpret as Periodic Bedrock Ridges (PBRs). Clues for a PBR origin result from ridge regularity, defect terminations, and the presence of preserved megaripples detaching from the PBRs. PBR orientation differs from superimposed transverse aeolian ridges pointing toward a major change in wind regime. Our results provide constrains on PBR formation mechanisms and offer indications on paleo winds that will be crucial for understanding the landing site geology.
Plain Language Summary
Oxia Planum on Mars is the landing site for the ExoMars 2022 mission. The region likely hosted a standing body of water, but the effect of the wind was also important in shaping the landscape. In this study, we first describe a set of linear ridges that, in our interpretation, were sculpted by the wind in a more recent past. We also show that the wind that formed the ridges (Periodic Bedrock Ridges) was blowing from a different direction than the ones that formed younger ripples on top, suggesting a complex geological history of wind erosion and deposition that will be further investigated during the ExoMars mission.
Key Points
We present the first evidence for a periodic bedrock ridge (PBRs) pattern from the ExoMars 2022 landing site
Formative paleowind directions are extrapolated from PBRs and transverse aeolian ridges
Evidence for an Amazonian change in the wind regime are provided
The T2K experiment measures muon neutrino disappearance and electron neutrino appearance in accelerator-produced neutrino and antineutrino beams. With an exposure of 14.7(7.6)×1020 protons on target ...in the neutrino (antineutrino) mode, 89 νe candidates and seven anti-νe candidates are observed, while 67.5 and 9.0 are expected for δCP=0 and normal mass ordering. The obtained 2σ confidence interval for the CP-violating phase, δCP, does not include the CP-conserving cases (δCP=0, π). The best-fit values of other parameters are sin2θ23=0.526−0.036+0.032 and Δm322=2.463−0.070+0.071×10−3 eV2/c4.
Background
Hemorrhagic cystitis (HC) is a common complication after hematopoietic allogeneic stem cell transplantation (HSCT) associated with intensity of the conditioning regimen, cyclophosphamide ...(Cy) therapy, and BK polyomavirus (BKPyV) infection.
Methods
We analyzed 33 consecutive haploidentical (haplo) HSCT recipients transplanted for hematologic diseases. Eleven patients had a previous transplant. Median follow‐up was 11 months. Graft‐versus‐host disease (GVHD) prophylaxis consisted of cyclosporine + mycophenolate mofetil and post‐HSCT Cy.
Results
Thirty‐two of 33 patients achieved neutrophil recovery. Cumulative incidence (CI) of platelet recovery was 65%. CI grade II–IV acute GVHD was 44%. Twenty patients developed HC in a median time of 38 days. CI of HC at day 180 was 62%. BKPyV was positive in blood and urine of 91% of patients at HC onset. HC resolved in 18/20 patients. Factors associated with HC were previous transplant (P = 0.01) and occurrence of cytomegalovirus reactivation before HC (P = 0.05). Grade II–IV acute GVHD was not associated with HC (P = 0.62). CI of day 180 viral infections was 73%. Two‐year overall survival (OS) was 50%; HC did not impact OS (P = 0.29).
Conclusion
The incidence of HC after haplo with post‐HSCT Cy is high and is associated with morbidity, especially in high‐risk patients such as those with a previous transplant history and with impaired immune reconstitution.
Abstract
The main source of systematic uncertainty on neutrino cross section measurements at the GeV scale originates from the poor knowledge of the initial flux. The reduction of this uncertainty to ...1% can be achieved through the monitoring of charged leptons produced in association with neutrinos. The goal of the ENUBET ERC project is to prove the feasibility of such a monitored neutrino beam. In this contribution, the final results of the ERC project, together with the complete assessment of the feasibility of its concept, are presented. An overview of the detector technology for a next generation of high precision neutrino-nucleus cross section measurements, to be performed with the ENUBET neutrino beam, is also given.
The ENUBET ERC project (2016–2021) is studying a narrow band neutrino beam where lepton production can be monitored at single particle level. For this purpose, the decay tunnel is instrumented with ...longitudinally segmented calorimeters. Three different specialized calorimeters have been designed and tested, two of which based on the shashlik calorimetric concept with a compact readout while the third is a less compact version with a lateral readout. All of the prototypes are composed of thick steel absorbers coupled to plastic scintillators. Regarding the shashlik modules, a matrix of 3 × 3 fibers runs transversely with a density of one fiber/cm2. The fibers are coupled individually to silicon photomultipliers mounted on a custom PCB allowing to reduce the dead zones between adjacent modules to an extremely small level compared to the “fiber bundling” configurations. This setup allows a very effective longitudinal segmentation and hence e/π separation. The second shashlik module is based on polysiloxane scintillators which come in liquid form, are poured around the fiber arrays and finally made solid with a thermal treatment. Finally, the lateral readout module, light is collected from both sides of each scintillator tile and the 10 fibers from the same UCM are bundled to a single SiPM. Here are discussed the results of test beams performed in 2016–2018 at the CERN-PS East Area and the characterization of SiPMs of different cell size (12μm and 15μm) before and after being exposed to neutron fluxes up to 1012/cm2 at the INFN-LNL CN accelerator facility.
Abstract
The ENUBET ERC project, also included in the CERN Neutrino Platform as NP06/ENUBET, is developing a new neutrino beam based on conventional techniques in which the flux and the flavor ...composition are known with unprecedented precision (
O
(1%)). Such a goal is accomplished monitoring the associated charged leptons produced in the decay region of the ENUBET facility. Positrons and muons from kaon decays are measured by a segmented calorimeter instrumenting the walls of the decay tunnel, while muon stations after the hadron dump can be used to monitor the neutrino component from pion decays. Furthermore, the narrow momentum width (<10%) of the beam provides a precise measurement (
O
(10%)) of the neutrino energy on an event by event basis, thanks to its correlation with the radial position of the interaction at the neutrino detector. ENUBET is therefore an ideal facility for a high precision neutrino cross-section measurement at the GeV scale, that could enhance the discovery potential of the next-generation of long baseline experiments. It is also a powerful tool for testing the sterile neutrino hypothesis and to investigate possible non-standard interactions.
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