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The study of clustering states in neutron-rich nuclei is an important subject of research in the field of nuclear physics, steadily growing in interest in the international scientific community. In this context, break-up reactions play an important role for the characterization of exotic states in radioactive light nuclei, like neutron halos around stable cores, α-clustering structures or exotic clusters. The CLIR (Clusters in Light Ion Reactions) experiment was performed at INFN - Laboratori Nazionali del Sud (LNS), aiming at the investigation of such states in light radioactive nuclei, by producing a radioactive beam at the FRIBs facility. Reaction products were detected by the CHIMERA multidetector, coupled with four telescopes of the FARCOS array. Calibrations of the tagging system and of the FARCOS telescopes have been performed, for which accurate procedures have been carried out. In this paper, results on the analysis will be presented. Moreover, a brief review on the new fragment separator FRAISE, currently under construction at LNS, will be given.

A project for an upgrade of the Superconducting Cyclotron is underway at INFN-LNS. One of the goals of this project is the production of RIBs (Radioactive Ion Beams) of high intensity. To reach this purpose, a dedicated facility consisting of a new fragment separator FRAISE (FRAgment In-flight SEparator) is ongoing, exploiting primary beams with a power up to ≈ 2-3 kW. The high intensity achievable with FRAISE requires the use of appropriate diagnostics and tagging systems that can operate also in a strong radioactive environment. In this framework, a R&D program has been started to develop the FRAISE facility as well as the diagnostics and the tagging systems.

The next years will see the completion of several new facilities at Istituto Nazionale di Fisica Nucleare – Laboratori Nazionali del Sud (LNS) opening up new possibilities in the fields of nuclear structure, nuclear dynamics, nuclear astrophysics and applications. These include a new line for high-intensity cyclotron beams, a new facility for in-flight production of radioactive ion beams, the PANDORA plasma trap for multidisciplinary studies and a high-power laser for basic science and applied physics. The nuclear physics community has organized a workshop to discuss the new physics opportunities that will be possible in the middle term (5–7 years) by employing state-of-the-art detection systems. A detailed discussion of the outcome from the workshop is presented in this report.

The development of new detectors based on Silicon Carbide (SiC) is currently a topic of interest within the scientific community. The significant features of SiC make it highly promising for detecting charged particles, neutrons, and \\(\\)/X radiation. In this framework, within the SAMOTHRACE (Sicilian Micro and Nano Technology Research and Innovation Center) ecosystem, an array of new-generation SiC detectors is under development, specifically designed for nuclear and medical investigations using radioactive ion beams. This paper describes the results obtained in the characterization of SiC prototypes regarding energy and timing measurements. A new method, based on coincidence data analysis, is employed to evaluate the timing performances of SiC detectors. The obtained results have been compared with tests performed using a micro-channel plate as a start detector reference for timing measurements.

Anemia associated with PNH is caused by hemolysis. The C5 inhibitor eculizumab blocks intravascular hemolysis, but anemia often persists owing to extravascular hemolysis. Pegcetacoplan, an inhibitor of C3, prevents extravascular hemolysis. After 16 weeks of treatment, hemoglobin increased nearly 4 g per deciliter in 41 patients treated with pegcetacoplan, and 85% no longer needed transfusion.

The standard busulfan–cyclophosphamide myeloablative conditioning regimen is associated with substantial non-relapse mortality in patients older than 40 years with acute myeloid leukaemia who are undergoing allogeneic stem-cell transplantation. Because the combination of busulfan plus fludarabine has been proposed to reduce non-relapse mortality, we aimed to compare this treatment with busulfan plus cyclophosphamide as a preparative regimen in these patients. We did an open-label, multicentre, randomised, phase 3 trial for patients with acute myeloid leukaemia at 25 hospital transplant centres in Italy and one in Israel. Eligible patients were aged 40–65 years, had an Eastern Cooperative Oncology Group performance status less than 3, and were in complete remission. Patients were randomly assigned 1:1 to receive intravenous busulfan plus cyclophosphamide or busulfan plus fludarabine. Treatment allocations were not masked to investigators or patients. Randomisation was done centrally via a dedicated web-based system using remote data entry, with patients stratified by donor type and complete remission status. Patients allocated to busulfan plus cyclophosphamide received intravenous busulfan 0·8 mg/kg four times per day during 2 h infusions for four consecutive days (16 doses from days −9 through −6; total dose 12·8 mg/kg) and cyclophosphamide at 60 mg/kg per day for two consecutive days (on days −4 and −3; total dose 120 mg/kg). Patients allocated to busulfan plus fludarabine received the same dose of intravenous busulfan (from days −6 through −3) and fludarabine at 40 mg/m2 per day for four consecutive days (from days −6 through −3; total dose 160 mg/m2). The primary endpoint was 1-year non-relapse mortality, which was assessed on an intention-to-treat basis; safety outcomes were assessed in the per-protocol population. This trial has been completed and is registered with ClinicalTrials.gov, number NCT01191957. Between Jan 3, 2008, and Dec 20, 2012, we enrolled and randomly assigned 252 patients to receive busulfan plus cyclophosphamide (n=125) or busulfan plus fludarabine (n=127). Median follow-up was 27·5 months (IQR 9·8–44·3). 1-year non-relapse mortality was 17·2% (95% CI 11·6–25·4) in the busulfan plus cyclophosphamide group and 7·9% (4·3–14·3) in the busulfan plus fludarabine group (Gray's test p=0·026). The most frequently reported grade 3 or higher adverse events were gastrointestinal events (28 [23%] of 121 patients in the busulfan plus cyclophosphamide group and 26 [21%] of 124 patients in the busulfan plus fludarabine group) and infections (21 [17%] patients in the busulfan plus cyclophosphamide group and 13 [10%] patients in the busulfan plus fludarabine group had at least one such event). In older patients with acute myeloid leukaemia, the myeloablative busulfan plus fludarabine conditioning regimen is associated with lower transplant-related mortality than busulfan plus cyclophosphamide, but retains potent antileukaemic activity. Accordingly, this regimen should be regarded as standard of care during the planning of allogeneic transplants for such patients. Agenzia Italiana del Farmaco.

In this randomized trial, eculizumab, a humanized monoclonal antibody against C5 that inhibits terminal complement activation, was compared with placebo as a treatment for paroxysmal nocturnal hemoglobinuria. The antibody stabilized hemoglobin levels, decreased the need for transfusions, and improved the quality of life by reducing intravascular hemolysis. Eculizumab stabilized hemoglobin levels, decreased the need for transfusions, and improved the quality of life by reducing intravascular hemolysis. (Figure courtesy of Alexion Pharmaceuticals.) Paroxysmal nocturnal hemoglobinuria (PNH), an uncommon form of hemolytic anemia, results from the clonal expansion of hematopoietic stem cells that have somatic mutations in the X-linked gene PIG-A . 1 , 2 PIG-A mutations cause an early block in the synthesis of glycosylphosphatidylinositol (GPI) anchors, which tether many proteins to the cell surface. Consequently, the blood cells in patients with PNH have a partial deficiency (type II) or a complete deficiency (type III) of GPI-linked proteins. Intravascular hemolysis is a prominent feature of PNH and is the consequence of the absence of the GPI-linked complement regulatory protein CD59. 3 , 4 CD59 blocks the . . .

Persistent hemolytic anemia and a lack of oral treatments are challenges for patients with paroxysmal nocturnal hemoglobinuria who have received anti-C5 therapy or have not received complement inhibitors. Iptacopan, a first-in-class oral factor B inhibitor, has been shown to improve hemoglobin levels in these patients. In two phase 3 trials, we assessed iptacopan monotherapy over a 24-week period in patients with hemoglobin levels of less than 10 g per deciliter. In the first, anti-C5-treated patients were randomly assigned to switch to iptacopan or to continue anti-C5 therapy. In the second, single-group trial, patients who had not received complement inhibitors and who had lactate dehydrogenase (LDH) levels more than 1.5 times the upper limit of the normal range received iptacopan. The two primary end points in the first trial were an increase in the hemoglobin level of at least 2 g per deciliter from baseline and a hemoglobin level of at least 12 g per deciliter, each without red-cell transfusion; the primary end point for the second trial was an increase in hemoglobin level of at least 2 g per deciliter from baseline without red-cell transfusion. In the first trial, 51 of the 60 patients who received iptacopan had an increase in the hemoglobin level of at least 2 g per deciliter from baseline, and 42 had a hemoglobin level of at least 12 g per deciliter, each without transfusion; none of the 35 anti-C5-treated patients attained the end-point levels. In the second trial, 31 of 33 patients had an increase in the hemoglobin level of at least 2 g per deciliter from baseline without red-cell transfusion. In the first trial, 59 of the 62 patients who received iptacopan and 14 of the 35 anti-C5-treated patients did not require or receive transfusion; in the second trial, no patients required or received transfusion. Treatment with iptacopan increased hemoglobin levels, reduced fatigue, reduced reticulocyte and bilirubin levels, and resulted in mean LDH levels that were less than 1.5 times the upper limit of the normal range. Headache was the most frequent adverse event with iptacopan. Iptacopan treatment improved hematologic and clinical outcomes in anti-C5-treated patients with persistent anemia - in whom iptacopan showed superiority to anti-C5 therapy - and in patients who had not received complement inhibitors. (Funded by Novartis; APPLY-PNH ClinicalTrials.gov number, NCT04558918; APPOINT-PNH ClinicalTrials.gov number, NCT04820530.).

Pregnancy has been discouraged in patients with paroxysmal nocturnal hemoglobinuria, a life-threatening hemolytic anemia, because of the heightened risk. However, in this study, eculizumab protected the mother and had no obvious adverse effect on the babies. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem-cell disorder that is characterized by chronic hemolysis, bone marrow failure, and venous thromboembolism. 1 – 3 Manifestations of the disease are related primarily to complement-mediated hemolysis. Patients with PNH present with a wide range of clinical symptoms, and if they do not receive specific treatment for the disorder, they can have a chronic, progressive illness and an increasing risk of death, primarily from thrombosis, over time (the median survival from the time of diagnosis ranges from 10 to 32 years). 1 – 3 Historically, the management of PNH during pregnancy has been challenging, and pregnancy . . .

Purpose Total knee arthroplasty is a successful procedure in treating subjects with end-stage knee osteoarthritis. The objective of this matched study was to evaluate subjective patient satisfaction and clinical and radiological outcomes in two groups of patients undergoing primary TKA using an identical third-generation design with different conformity in the polyethylene insert. Methods One hundred consecutive patients undergoing TKA because of knee osteoarthritis were randomized in two matched groups. Group A included 50 Posterior-Stabilized (PS) implants, while group B included 50 Medially Congruent (MC) implants. The surgical technique was identical: gap balancing in extension and measured resection in flexion; cruciate ligaments were always removed; the coronal alignment followed the mechanical axis and the tibial slope was set at 3° in the PS group and 5° in the MC. Oxford Knee Score (OKS) and Knee Society Score (KSS) were assessed preoperatively and at 2 year minimum follow-up. Two-sample T test statistical analysis was performed. Results All patients were available at final follow-up: there were no preoperative statistical differences between the two groups in the average preoperative ROM (PS 112°, MC 108°; n.s.), average preoperative KSS (PS 64.4, MC 63.7; n.s.), average preoperative OKS (PS 19.6; MC 19.0; n.s.), and average BMI (PS 34.40, MC 34.60; n.s.). At final follow-up, there were no statistical differences between the two groups in the average OKS (PS 40,5; MC 41.1; n.s.) and in the average KSS (PS 161,5, MC 165,7; n.s.). We found a statistically but not clinically significant difference at final ROM: the average maximum active flexion was 120° in the PS group and 123° in the MC group (s.s.). Conclusion This study evaluated two biomechanically different polyethylene inserts in the same TKA design, showing that reducing the level of intra-articular conformity had minimal effects on PROMs and objective short-term clinical results but a potentially beneficial effect on ROM. This study suggests that, once a satisfactory intra-operative stability is obtained, the minimal level of constraint should be used. Level of evidence III.