First optical validation of a Schwarzschild Couder telescope: the ASTRI SST-2M Cherenkov telescope

The Cherenkov Telescope Array (CTA) represents the most advanced facility designed for Cherenkov Astronomy. ASTRI SST-2M has been developed as a demonstrator for the Small Size Telescope in the context of the upcoming CTA. Its main innovation consists in the optical layout which implements the Schwarzschild-Couder configuration and is fully validated for the first time. The ASTRI SST-2M optical system represents the first qualified example for two mirrors telescope for Cherenkov Astronomy. This configuration permits to (i) maintain a high optical quality across a large FoV (ii) de-magnify the plate scale, (iii) exploit new technological solutions for focal plane sensors. The goal of the paper is to present the optical qualification of the ASTRI SST-2M telescope. The qualification has been obtained measuring the PSF sizes generated in the focal plane at various distance from the optical axis. These values have been compared with the performances expected by design. After an introduction on the Gamma Astronomy from the ground, the optical design and how it has been implemented for ASTRI SST-2M is discussed. Moreover the description of the setup used to qualify the telescope over the full field of view is shown. We report the results of the first--light optical qualification. The required specification of a flat PSF of $\sim 10$ arcmin in a large field of view ~10 deg has been demonstrated. These results validate the design specifications, opening a new scenario for Cherenkov Gamma ray Astronomy and, in particular, for the detection of high energy (5 - 300 TeV) gamma rays and wide-field observations with CTA.Comment: 6 pages, 5 figure

Sulfide-, fluorite-, barite-bearing siliceous "crusts" related to unconformity surfaces of different ages in Pyrenees and Alps: a new model in carbonate-hosted deposits?

Wumerous stratabound sulfide-, barite-, fluonte-bearing siliceous crusts, from dm to some tens of in thick, occur over large areas of the Alpine belt, ;.e. the Alps and the Pyrenees. They are linked to unconformlty landscapes evolved on various carbonate units of Paieozoic and Triassic sedimentq sequences. Since the study mineralizations constitute the transition between the underlying carbonates and the overlying detrital units, they can be considered as an independent lithostratigraphic units that record a particular metalogenetic process not only in the alpine chains but worldwide. These mineralizations exhibit several morphologies: tabular concordant with the unconfonnities bodies, columnar bodies, karstic cavity-fillings, laminites and veins. In addition, the study deposits are clearly affected by remobilization process occuned during diagenesis or metamorphism. Such processes are responsible for masking the occunence of the breccia/conglomerate typically located at the base of the orebodies.Although the study mineralizations have usually been included in MVT deposit class, constrastirig differences between their diagnostic features and those of MVT mineralizations, suggest that the inclusion of the mineralized crust deposits in the MVT group seem incorrect.These peculiar ore-bearing quartz-crusts, persistent over large areas and showing an independent and distinct character and constituting an important marker for some sedimentary sequences of different ages in Alpine belts, allow the authors to define a new metallogenic model named as "crust-type" (CT) deposits. Comparable mineralization in other geotectonic environments outside Alpine belts point out to CT deposits being a worldwide significant metallogenic event

Sivelestat relieves respiratory distress refractory to dexamethasone in all-trans retinoic acid syndrome: a report of two cases

Kawasaki K, Akaike H, Miyauchi A, Ouchi K. Sivelestat relieves respiratory distress refractory to dexamethasone in all-trans retinoic acid syndrome: a report of two cases. Treatment with all-trans retinoic acid (ATRA) improves the prognosis of patients with acute promyelocytic leukemia (APL), but ATRA syndrome may occur as a possible fatal side effect, especially in cases refractory to medication or involving pulmonary hemorrhage. We describe two patients with APL who suffered from intracranial hemorrhage. The first patient was a 16-yr-old girl who was treated with ATRA and then developed respiratory distress refractory to treatment with dexamethasone combined with anthracycline-cytarabine cytoreduction therapy. Treatment with Sivelestat, a small molecule inhibitor of neutrophil elastase, achieved rapid improvement in oxygenation and chest radiograph findings, and the patient has been in complete remission for 24 months. The second patient was a 10-yr-old boy in whom pulmonary hemorrhage developed following administration of ATRA, dexamethasone and cytoreduction therapy. Aspiration and administration of Sivelestat improved oxygenation and he remained stable. Hematological improvement was also achieved, but the patient died of brain dysfunction because of cerebral edema accompanied by intracranial bleeding. The two cases suggest that Sivelestat may be effective as an additional agent in the treatment of refractory ATRA syndrome, and, therefore, prospective randomized studies of treatment protocols are warranted

von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment

Von Willebrand's disease (VWD) is an autosomally inherited bleeding disorder caused by a deficiency or abnormality of von Willebrand factor (VWF). VWF is a multimeric adhesive protein which plays an important role in primary hemostasis by promoting platelet adhesion to the subendothelium at sites of vascular injury and platelet-platelet interactions in high shear-rate conditions. It is also the carrier of factor VIII (FVIII), thus indirectly contributing to the coagulation process. VWD has a prevalence of about 1% in the general population, but the figure for clinically relevant cases is lower (about 100/million inhabitants). Bleeding manifestations are heterogeneous: mucosal bleeding is typical of all VWD cases but hemarthrosis and hematomas may also be present when FVIII levels are low. Most cases appear to have a partial quantitative deficiency of VWF (type 1 VWD) with variable bleeding tendency, whereas qualitative variants (type 2 VWD), due to a dysfunctional VWF, are clinically more homogeneous. Type 3 VWD is rare and the patients have a moderate to severe bleeding diathesis because of the virtual absence of VWF, and a recessive pattern of inheritance. The diagnosis of VWD, especially type I, may be difficult, because the laboratory phenotype is highly heterogeneous and is confounded by the fact that factors outside the VWF gene (e.g., blood group) influence VWF levels. An array of tests is usually required to characterize the VWD types of the disorder and establish the best treatment modality. The aim of treatment is to correct the dual defect of hemostasis, i.e. abnormal coagulation expressed by low levels of FVIII and abnormal platelet adhesion expressed by the prolonged bleeding time (BT). Desmopressin (DDAVP) is the treatment of choice for type 1 VWD because it corrects the FVIII/VWF levels and the prolonged BT in the majority of cases. In type 3 and in severe forms of type 1 and 2 VWD, DDAVP is not effective and for these patients plasma virally-inactivated concentrates containing FVIII and VWF are the mainstay of treatment. These concentrates are clinically effective and safe, although they do not always correct the BT

Autoimmune cytopenias in chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is frequently complicated by secondary autoimmune cytopenias (AIC) represented by autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia and autoimmune granulocytopenia. The distinction of immune cytopenias from cytopenias due to bone marrow infiltration, usually associated with a worse outcome and often requiring a different treatment, is mandatory. AIHA and ITP are more frequently found in patients with unfavorable biological risk factors for CLL. AIC secondary to CLL respond less favorably to standard treatments than their primary forms, and treating the underlying CLL with chemotherapy or monoclonal antibodies may ultimately be necessary

Expected performance of the ASTRI-SST-2M telescope prototype

ASTRI (Astrofisica con Specchi a Tecnologia Replicante Italiana) is an Italian flagship project pursued by INAF (Istituto Nazionale di Astrofisica) strictly linked to the development of the Cherenkov Telescope Array, CTA. Primary goal of the ASTRI program is the design and production of an end-to-end prototype of a Small Size Telescope for the CTA sub-array devoted to the highest gamma-ray energy region. The prototype, named ASTRI SST-2M, will be tested on field in Italy during 2014. This telescope will be the first Cherenkov telescope adopting the double reflection layout in a Schwarzschild-Couder configuration with a tessellated primary mirror and a monolithic secondary mirror. The collected light will be focused on a compact and light-weight camera based on silicon photo-multipliers covering a 9.6 deg full field of view. Detailed Monte Carlo simulations have been performed to estimate the performance of the planned telescope. The results regarding its energy threshold, sensitivity and angular resolution are shown and discussed.Comment: In Proceedings of the 33rd International Cosmic Ray Conference (ICRC2013), Rio de Janeiro (Brazil). All CTA contributions at arXiv:1307.223

Old and new prognostic factors in acute myeloid leukemia with deranged core-binding factor beta.

Acute myeloid leukemia (AML) with deranged core-binding factor beta (CBF\u3b2) is usually associated with a favorable prognosis with 50-70% of patients cured using contemporary treatments. We analyzed the prognostic significance of clinical features on 58 patients with CBF\u3b2-AML aged 6460 years. Increasing age was the only predictor for survival (P <0.001), with an optimal cut-point at 43 years. White blood cells (WBCs) at diagnosis emerged as an independent risk factor for relapse incidence (P\u2009=\u20090.017), with 1.1% increase of hazard for each 1.0 7 109 /L WBC increment. KIT mutations lacked prognostic value for survival and showed only a trend for relapse incidence (P\u2009=\u20090.069)