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Therefore, the cost-effective rN212/GO composite membrane is a promising alternative to suppress migration of vanadium ions across the membrane to set up VRFB cells with better performances. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.AIHA is rare in the general population and associated with a mortality of 8%. In contrast, AIHA occurs in up to 12.2% of cases after intestinal transplantation and is associated with mortality up to 50%. Treatment entails a "step-up" approach including corticosteroids, IvIg, plasmapheresis, and rituximab. However, AIHA after transplantation often is refractory to this strategy, contributing to a poor outcome. We describe a child with microvillous inclusion disease who developed AIHA 1 year after multivisceral transplantation that was refractory to standard therapy and was subsequently treated with bortezomib.We observed remission of AIHA within 1 week after the start of bortezomib. Bortezomib was associated with transient diarrhea, leucopenia, and elevated liver enzymes. Three years later, he remains in remission without important complications. Published data on bortezomib for autoimmune cytopenias outside SOT are discussed. This is the first report to support bortezomib as an important therapeutic alternative for AIHA after SOT. The occurrence and treatment of AIHA after SOT, and specifically intestinal transplantation, should be the subject of future registry studies to collect additional experience and explore the optimal therapeutic approach. © 2020 Wiley Periodicals, Inc.Interfaces play an important role in enhancing the energy conversion performance of dye-sensitized solar cells (DSCs). The interface effects have been studied by many techniques, but almost all studies only performed on a portion of DSC, rather than on a complete solar cell. Hence, monitoring the interface evolution of a DSC is still very challenging. Here, in situ/operando resonance Raman (RR) spectroscopic analyses were carried out to monitor the dynamic observations of the photovoltaic conversion process from a DSC. We observed the creation of new species (i.e., polyiodide and iodine aggregates) in the photosensitization process. We also successfully identified molecular-scale dynamic evidence that the bands from the C=C of 2,2'-bipyridyl (bpy), the S=C=N of NCS ligand and photochemical products show reasonably strong intensity and frequency changes, which clearly demonstrates that they are important pathways of charge separation. Furthermore, RR spectroscopy also be applicable to quickly evaluate the performance of DSCs. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.OBJECTIVE We sought to investigate the effectiveness and safety of dose reduction of tumour necrosis factor inhibitor (TNFi) therapy in the treatment of axial spondyloarthritis (axSpA), compared to usual care. METHODS We searched CENTRAL, Embase, MEDLINE and trial registries. We screened, extracted data, and assessed risk of bias in duplicate. Data were pooled using random-effects models; subgroup analyses were performed for type of TNFi, prior TNFi exposure and follow-up duration. Outcomes of interest were Assessment in SpondyloArthritis international Society (ASAS) response and remission criteria, disease activity, relapse and safety. RESULTS We included six randomized trials with 747 participants (442 AS and 305 nr-axSpA). Compared to standard dose, there were fewer ASAS40 (risk ratio [RR] 0.62, 95% confidence interval [CI] 0.49 - 0.78) and ASAS partial remission (RR 0.17, 95% CI 0.06 - 0.46) events with the reduced dose. There was a mean increase in the Bath Ankylosing Spondylitis Disease Activity Index score (mean difference [MD] 0.35, 95% CI 0.10 - 0.60), and no difference in C-reactive protein levels (MD 0.16, 95% CI -0.76 - 1.07) with the reduced dose. There were more disease flares/relapses (RR 1.73, 95% CI 1.32 - 2.27) with the reduced dose. There were no differences in infection rates (incidence rate ratio [IRR] 0.98, 95% CI 0.76 - 1.25) or injection/infusion reactions (IRR 0.71, 95% CI 0.42 - 1.19). CONCLUSION Patients with axSpA may experience little to no clinical benefit from reduction of TNFi therapy. Maintaining the standard dose probably improves the sustained effect on disease activity and helps to prevent disease flare. This article is protected by copyright. All rights reserved.INTRODUCTION Advances in genomic sequencing have facilitated the sequencing of genes associated with disorders of haemostasis. The identification of variants within genes and access to curated data incorporating structural, functional, evolutionary as well as phenotypic data has become increasingly important in order to ascribe pathogenicity. AIM The European Association for Haemophilia and Allied Disorders (EAHAD) Coagulation Factor Variant Database Project aims to provide a single port of entry to a web-accessible resource for variants in genes involved in clinical bleeding disorders. RESULTS New databases have evolved from previously developed single gene variant coagulation database projects, incorporating new data, new analysis tools and a new common database architecture with new interfaces and filters. These new databases currently present information about the genotype, phenotype (laboratory and clinical) and structural and functional effects of variants described in the genes of factor (F) VII (F7), FVIII (F8), FIX (F9) and von Willebrand factor (VWF). CONCLUSION The project has improved the quality and quantity of information available to the haemostasis research and clinical communities, thereby enabling accurate classification of disease severity in order to make assessments of likely pathogenicity. © 2020 The Authors. Haemophilia published by John Wiley & Sons Ltd.NaNO 3 promoted MgO composite materials are prepared and their ability to sorb CO 2 at a concentration relevant to CO 2 capture from flue gas is explored. The uptake kinetics and capacities of sorbents of different NaNO 3 MgO ratios are measured at intermediate temperatures ranging from 230 °C - 300 °C. The sorbent with a NaNO 3 MgO ratio of 0.10 shows the highest 12 h sorption capacity among sorbents of different NaNO 3 loading, and the highest sorption capacity of 11.2 mmol CO 2 /g is observed 260 °C for this material. Intriguingly, an induction period is observed in the initial stage of CO 2 sorption. In-situ XRD analysis, in-situ FTIR spectroscopy, and a comparison of the CO 2 sorption behavior under simulated flue gas conditions vs literature reports employing pure CO 2 indicate that the sorption of CO 2 occurs through nucleation of MgCO 3 crsytallites in the material. The new data reported here also indicate that the concentration of CO 2 within the molten medium of NaNO 3 , which is affected by both the solubility of CO 2 in molten NaNO 3 and the partial pressure of CO 2 in the surrounding atmosphere, has a critical impact on the length of the induction period. It is shown that a partially desorbed sample after sorption of CO 2 shows much improved sorption kinetics in the next cycle, and sorbs and desorbs CO 2 over multiple cycles at isothermal conditions by switching the feed gas from CO 2 to inert gas. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.CO2 electroreduction has gained significant interest. However, fabricating cost-effective non-precious metal electrocatalysts that can selectively convert CO2 to a specific product remains highly challenging. Herein, ordered mesoporous carbon (OMC) confined Pb-based materials with co-existence of Pb0 and PbO (Pb/PbO@OMC) were constructed for CO2 electroreduction to CO. Interestingly, the activity and selectivity of the Pb/PbO@OMC varied with the molar ratio of Pb0/PbO. The material calcined at 800 oC (Pb/PbO@OMC-800) with a Pb0/PbO ratio of 0.58 provided the best result with CO as the only carbon-based product, and the Faradaic efficiency of CO could reach 98.3% at a high current density of 41.3 mA cm-2. Detailed studies indicated that Pb0, PbO, and OMC cooperated well to enhance the performance of Pb/PbO@OMC-800, which mainly originated from the proper interface of Pb0 and PbO, higher electrochemical active surface area, and faster electron transfer to form CO2•- intermediate. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.Short telomere syndromes are a heterogenous spectrum of disorders leading to premature cellular aging. These may involve bone marrow failure, adult-onset idiopathic pulmonary fibrosis, and liver disease, and classical entities such as dyskeratosis congenita. We report a patient who presented with common variable immunodeficiency at 3 years of age and autoimmune cytopenias at 8 years of age. He was found to have short telomeres, and genetic testing confirmed a hemizygous mutation NM_001363.4 c.-142C > G in DKC1 gene. He subsequently developed cirrhosis with severe portal hypertension and hepatopulmonary syndrome, prompting liver transplantation at 11 years of age. He remains well 10 years after transplant with no progression of bone marrow failure or progressive lung disease. GW3965 In conclusion, short telomere syndromes should be considered as a potential cause of pediatric liver disease of unknown etiology, and in severe cases, isolated liver transplantation may be both appropriate and successful. © 2020 Wiley Periodicals, Inc.BACKGROUND For better application in human forensic cases and population genetics research, it is imperative to investigate the genetic characteristics of Guanzhong Han population using enhanced Y-chromosomal short tandem repeats (Y-STR) detecting system with higher discriminating power than previous ones. METHODS In this study, 38 Y-STRs were profiled in 430 unrelated Chinese Han male individuals from Guanzhong region of Shaanxi Province, Northwest China, using the Yfiler™ Platinum PCR Amplification Kit. Haplotype frequencies and forensic parameters were calculated. Comprehensive population comparisons with geographically/ethnically different populations in China and other worldwide countries were performed. RESULTS A total of 422 different haplotypes were observed with the overall haplotype diversity (HD), discriminatory power (DC) and haplotype match probability (HMP) were 0.9999, 0.9814, and 0.0024, respectively. Guanzhong Han showed genetically affinity with Han ethnicity from Shanxi and Henan provinces, while far distant from Tibetan populations. CONCLUSION This study offered a unique insight into Guanzhong Han population, the 38 Y-STRs included in the the Yfiler™ Platinum system are highly polymorphic and informative and can be used for forensic practice and human genetic research. © 2020 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.The replacement of carbon with nitrogen can affect the aromaticity of organic rings. Nucleus-independent chemical shift (NICS) calculations at the center of the aromatic π-systems reveal that incorporating nitrogen into 5-membered heteroaromatic dienes has only a small influence on aromaticity. In contrast, each nitrogen incorporated into benzene results in a sequential and substantial loss of aromaticity. The contrasting effects of nitrogen-substitution in 5-membered dienes and benzene are reflected in their Diels-Alder reactivities as dienes. 1,2-Diazine experiences a 1011-fold increase in reactivity upon nitrogen-substitution at the 4- and 5-positions, whereas a 5-membered heteroaromatic diene, furan, experiences a comparatively incidental 102-fold increase in reactivity upon nitrogen-substitution at the 3- and 4-positions. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.
Homepage: https://www.selleckchem.com/products/gw3965.html
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