Microwave-assisted protocol for squalene isolation and conversion from oil-deodoriser distillates

Aiming to design a green and efficient protocol for squalane production from low-cost biomasses, a practical and scalable procedure for squalene extraction and hydrogenation to squalane is presented herein. The oil-deodoriser distillates that are produced by the vegetal-oil production chain are a renewable and cheap source of squalene. We were able to isolate an enriched fraction containing 89.0% of pure squalene (yield 55.4%) from a matrix containing about 2% squalene. Efficient microwave- assisted esterification under heterogeneous catalysis enabled the separation of fatty-acid methyl esters (FAME) via vacuum distillation. The residue was purified by flash-chromatography on a C-18 silica column using MeOH/H2O/2-propanol as the mobile phase. Finally, squalene was hydrogenated to the more stable squalane in a pressure-resistant microwave reactor. The reaction was performed over a Pd/C catalyst in EtOH, and even in solvent-free conditions, and was optimised using commercial squalene (5 bar of H2 at 100°C for 1 h)

Sustainable isosorbide production by a neat one-pot MW-assisted catalytic glucose conversion

In the context of exploitation of new biomass-derived platform chemicals, isosorbide (1,4:3,6-dianhydro-D-sorbitol), obtained by the two-fold dehydration of sorbitol, is gaining increasing interest in several potential industrial applications. Seeking for more sustainable, efficient, and economically competitive green processes, the use of heterogeneous catalysts under microwave (MW) irradiation has been adopted for the development of a neat one-pot process from glucose. MW-assisted catalytic processes have shown the potential to reduce the reaction time and improve the selectivity, due to the interaction of MW with the reaction medium through the production of hot spots on the catalyst surface. Ru/C, Ru/Al2O3 and Ru/TiO2 were tested for glucose hydrogenation to sorbitol, while the dehydration step was favored by the addition of beta Zeolites (360:1 SiO2:Al2O3) allowing high isosorbide selectivity (>85 %). An extended structural and morphological characterization before and after the catalytic tests allowed to establish structure-activity relationships. Yields up to 47.1 % have been obtained directly from glucose in 1.5 h, achieving a considerable reduction of reaction time without the use of a solvent. thus paving the way for further investigations on biomass conversion into value-added products. With this aim, direct isosorbide production from milled cellulose was investigated. While the isosorbide yields still need to be improved, the dual role of formic acid both as acid catalyst for cellulose hydrolysis and H-donor for the reduction step was promisingly clarified

The optical head of the EnVisS camera for the Comet Interceptor ESA mission: Phase 0 study

EnVisS (Entire Visible Sky) is an all-sky camera specifically designed to fly on the space mission Comet Interceptor. This mission has been selected in June 2019 as the first European Space Agency (ESA) Fast mission, a modest size mission with fast implementation. Comet Interceptor aims to study a dynamically new comet, or interstellar object, and its launch is scheduled in 2029 as a companion to the ARIEL mission. The mission study phase, called Phase 0, has been completed in December 2019, and then the Phase A study had started. Phase A will last for about two years until mission adoption expected in June 2022. The Comet Interceptor mission is conceived to be composed of three spacecraft: spacecraft A devoted to remote sensing science, and the other two, spacecraft B1 and B2, dedicated to a fly-by with the comet. EnVisS will be mounted on spacecraft B2, which is foreseen to be spin-stabilized. The camera is developed with the scientific task to image, in push-frame mode, the full comet coma in different colors. A set of ad-hoc selected broadband filters and polarizers in the visible range will be used to study the full scale distribution of the coma gas and dust species. The camera configuration is a fish-eye lens system with a FoV of about 180°x45°. This paper will describe the preliminary EnVisS optical head design and analysis carried out during the Phase 0 study of the mission

Hematopoietic Cell Transplantation Cures Adenosine Deaminase 2 Deficiency: Report on 30 Patients.

Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis nodosa, lacunar ischemic strokes, and intracranial hemorrhages), immunodeficiency, lymphoproliferation, immune cytopenias, and bone marrow failure (BMF). Tumor necrosis factor (TNF-α) blockade is the treatment of choice for the vasculopathy, but often fails to reverse refractory cytopenia. We aimed to study the outcome of hematopoietic cell transplantation (HCT) in patients with DADA2. We conducted a retrospective study on the outcome of HCT in patients with DADA2. The primary outcome was overall survival (OS). Thirty DADA2 patients from 12 countries received a total of 38 HCTs. The indications for HCT were BMF, immune cytopenia, malignancy, or immunodeficiency. Median age at HCT was 9 years (range: 2-28 years). The conditioning regimens for the final transplants were myeloablative (n = 20), reduced intensity (n = 8), or non-myeloablative (n = 2). Donors were HLA-matched related (n = 4), HLA-matched unrelated (n = 16), HLA-haploidentical (n = 2), or HLA-mismatched unrelated (n = 8). After a median follow-up of 2 years (range: 0.5-16 years), 2-year OS was 97%, and 2-year GvHD-free relapse-free survival was 73%. The hematological and immunological phenotypes resolved, and there were no new vascular events. Plasma ADA2 enzyme activity normalized in 16/17 patients tested. Six patients required more than one HCT. HCT was an effective treatment for DADA2, successfully reversing the refractory cytopenia, as well as the vasculopathy and immunodeficiency. HCT is a definitive cure for DADA2 with > 95% survival

Coronavirus disease 2019 in patients with inborn errors of immunity: an international study

BACKGROUND:There is uncertainty about the impact of SARS-CoV-2 infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe COVID-19. This is relevant not only for these patients but also the general population, as studies of IEIs can unveil key requirements for host defense. OBJECTIVE:Describe the presentation, manifestations and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2. METHODS:An invitation to participate in a retrospective study was distributed globally to scientific, medical and patient societies involved in the care and advocacy for patients with IEI. RESULTS:We gathered information on 94 IEI patients with SARS-CoV-2 infection. Median age was 25-34 years. 53 patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) auto-inflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required non-invasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 requiring invasive ventilation, and 3 extra corporeal membrane oxygenation. Nine patients (seven adults, two children) died. CONCLUSIONS:This study demonstrates that (1) >30% of IEI patients had mild COVID19, and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect IEI patients, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are non-redundant or redundant for protection against SARS-CoV-2.Isabelle Meyts, Giorgia Bucciol, Isabella Quinti, Bénédicte Neven, Alain Fischer, Elena Seoan