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No increased risk for inhibitor development was found for any type of FVIII concentrate; either when comparing recombinant FVIII concentrates to plasma-derived FVIII concentrates (adjusted odds ratio 0·96, 95% confidence interval (CI) 0·36-2·52) or for specific types of FVIII concentrates. © 2020 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.BACKGROUND Pea (Pisum sativum) is a prevalent cool season crop that produces seeds valued for high protein content. Modern cultivars have incorporated several traits that improved harvested yield. However, progress toward improving seed quality has received less emphasis, in part due to the lack of tools for easily and rapidly measuring seed traits. In this study we evaluated the accuracy of single-seed near-infrared spectroscopy (NIRS) for measuring pea seed weight, protein, and oil content. A total of 96 diverse pea accessions were analyzed using both single-seed NIRS and wet chemistry methods. To demonstrate field relevance, the single-seed NIRS protein prediction model was used to determine the impact of seed treatments and foliar fungicides on protein content of harvested dry peas in a field trial. RESULTS External validation of Partial Least Squares (PLS) regression models showed high prediction accuracy for protein and weight (R2  = 0.94 for both) and less accuracy for oil (R2  = 0.74). Single seed weight was weakly correlated with protein and oil content in contrast to previous reports. In the field study, the single-seed NIRS predicted protein values were within 10 mg g-1 of an independent analytical reference measurement and were sufficiently precise to detect small treatment effects. CONCLUSION The high accuracy of protein and weight estimation show that single-seed NIRS could be used in the dual selection of high protein, high weight peas early in the breeding cycle allowing for faster genetic advancement toward improved pea nutritional quality. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.Since their discovery, the orphan nuclear receptors constitutive androstane receptor (CAR) and pregnane X receptor (PXR) have been regarded as master regulators of drug disposition and detoxification mechanisms that regulate the metabolism and transport of endogenous mediators and xenobiotics in organs including the liver, intestine and brain. HS-173 However, with proposals of new physiological functions for CAR and PXR, there is increasing interest in the role of these receptors in influencing brain function. This review will summarize key findings regarding the expression and function of CAR and PXR in the brain, hereby highlighting the need for further research in this field. This article is protected by copyright. All rights reserved.Recent genome-wide analyses of rare and common sequence variations have brought greater clarity to the genetic architecture of pulmonary arterial hypertension and implicated novel genes in disease development. Transcriptional signatures have been reported in whole lung tissue, pulmonary vascular cells and peripheral circulating cells. High-throughput platforms for plasma proteomics and metabolomics have identified novel biomarkers associated with clinical outcomes and provided molecular instruments for risk assessment. There are methodological challenges to integrating these datasets, coupled to statistical power limitations inherent to the study of a rare disease, but the expectation is that this strategy will reveal novel druggable targets and biomarkers that will open the way to personalised medicine. Here we review the current state-of-the-art and future promise of “omics” in the field of translational medicine in pulmonary arterial hypertension. This article is protected by copyright. All rights reserved.Capping protein regulator and myosin 1 linker 2 (CARMIL2) deficiency is characterized by impaired T cell activation, which is attributed to defective CD28-mediated co-signaling. Herein, we aimed to analyze the effect of exogenous interleukin (IL)-2 on in-vitro T cell activation and proliferation in a family with CARMIL2 deficiency. This study included four children (one male and three females; 2.5-10 years old at presentation). The patients presented with inflammatory bowel disease and recurrent viral infections. Genetic analysis revealed a novel homozygous 25-bp deletion in CARMIL2. Immunoblotting demonstrated the absence of CARMIL2 protein in all four patients and confirmed the diagnosis of CARMIL2 deficiency. T cells were activated in-vitro with addition of IL-2 in different concentrations. CD25 and interferon-γ (IFNγ) levels were measured after 48 h and 5 days of activation. CD25 surface expression on activated CD8+ and CD4+ T cells was significantly diminished in all patients compared to healthy controls. Additionally, CD8+ T cells from all patients demonstrated significantly reduced IFNγ production. When cells derived from CARMIL2-deficient patients were treated with IL-2, CD25 and IFNγ production increased in a dose-dependent manner. T cell proliferation, as measured by Cell Trace-Violet, was impaired in one patient and it was also rescued with IL-2. In conclusion, we found that IL-2 rescued T cell activation and proliferation in CARMIL2-deficient patients. Thus, IL-2 should be further studied as a potential therapeutic modality for these patients. This article is protected by copyright. All rights reserved.BACKGROUND Traditional chemical methods were mainly used to evaluate the total antioxidant activity of essential oils. How to determine the bioactivity of each compound in mixtures is an interesting research topic. Nowadays, an ultra-fast gas chromatography electronic nose (E-nose) has been gradually used in the detection of volatile compounds, but the screening of the active components of essential oils has not been reported. E-nose coupled with chemical methodology was established using the essential oil from rosemary (EOR) as a specific application example. The proposed method can both identify the chemical constituents of EOR and quickly screen the antioxidant by comparing the change of chromatographic peak area of every component in EOR before and after reaction with free radicals. RESULTS Among all chemical constituents in EOR, verbenone, eucalyptol and o-cymene showed the strongest scavenging abilities in 1,1′-diphenyl-2-picrylhydrazine (DPPH·), 2,2′-azino-bis(3-ethyl-benzothiazoline-6-sulphonate) (ABTS·+ ) and hydroxyl (·OH) radicals, respectively, with scavenging rates of 67.