We report the synthesis of a unique cubic metal-organic framework (MOF), Fe-HHTP-MOF, comprising hexahydroxytriphenylene (HHTP) supertetrahedral units and FeIII ions, arranged in a diamond topology. selleck inhibitor The MOF is synthesized under solvothermal conditions, yielding a highly crystalline, deep black powder, with crystallites of 300-500 nm size and tetrahedral morphology. Nitrogen sorption analysis indicates a highly porous material with a surface area exceeding 1400 m2  g-1 . Furthermore, Fe-HHTP-MOF shows broadband absorption from 475 up to 1900 nm with excellent absorption capability of 98.5 % of the incoming light over the visible spectral region. Electrical conductivity measurements of pressed pellets reveal a high intrinsic electrical conductivity of up to 10-3  S cm-1 . Quantum mechanical calculations predict Fe-HHTP-MOF to be an efficient electron conductor, exhibiting continuous charge-carrier pathways throughout the structure. ALD is a rare X-linked peroxisomal metabolic disorder with many distinct phenotypes of disease that emerge on a wide scale from adrenal insufficiency to fatal cALD which progresses to a vegetative state within a few years. Currently, HSCT is the only treatment method known to stabilize disease progression in patients with cALD. In this study, we aim to report our HSCT experience in patients with cALD and the factors that determine the success of HSCT, as a single-center experience. The study cohort involves 23 boys with cALD and three patients with ALD trait and new-onset abnormal behavior who underwent allogeneic HSCT between January 2012 and September 2019 in our transplantation center. Loes scoring, NFS, scale and MFD were performed for evaluating the severity of the cerebral disease. The study cohort was divided into two groups according to baseline NFS and Loes score early-stage (NFS≤1 and Loes score <9) and advanced stage (NFS>1 or Loes score ≥9). The pretransplant stage of disease impacted both OS and MFD-free survival. The estimated OS and MFD-free survival at 3years in patients with advanced disease were 46.1% (95% CI 19.0-73.2) and 23.1% (95% CI 0.2-46.0), respectively, and all patients with the early disease were alive (p .004) and MFD-free (p<.001) at 3years. This study demonstrated that early HSCT is vital in patients with cALD. The early-stage disease had a significant survival advantage and free from disease progression after HSCT.This study demonstrated that early HSCT is vital in patients with cALD. The early-stage disease had a significant survival advantage and free from disease progression after HSCT. Data on outcome in persons with haemophilia B (PwHB) are limited and mainly extrapolated from studies of haemophilia A (HA). To characterize treatment outcomes in persons with severe HB in the Nordic region, with a focus on joint health, compared with matched controls with HA. PwHB attending haemophilia centres in Denmark, Finland, Norway and Sweden were enrolled and matched with controls with HA. Joint assessment using Haemophilia Joint Health Score (HJHS) and ultrasound according to Haemophilia Early Arthropathy Detection protocol (HEAD-US) was conducted. Adherence was evaluated using the Validated Haemophilia Regimen Treatment Adherence Scale (VERITAS). Seventy-nine males with HB, with median age of 30years (range 1-75), were enrolled. Eleven patients (14%) had a history of or current inhibitor. Twenty-nine PwHB (37%) reported joint bleeds during the prior year, and 35% had previously undergone joint surgery. Ninety-five per cent were on prophylaxis, and 70% used recombinant concentrates, with a median factor consumption of 3,900IU/kg/year for standard half-life products. Only two patients had a VERITAS score corresponding to 'non-adherence'. Joint health, assessed with HJHS, showed a significant lower score among PwHB compared with HA controls, explained by a difference in the 18-49 age group, without observed differences in older or younger subgroups. The HEAD-US scores were overall low. The Nordic cohort of PwHB is well treated by prophylaxis, but the goal of zero bleeds for all is not reached. Our findings suggest that patients with severe HB suffer from a milder arthropathy than patients with severe HA.The Nordic cohort of PwHB is well treated by prophylaxis, but the goal of zero bleeds for all is not reached. Our findings suggest that patients with severe HB suffer from a milder arthropathy than patients with severe HA.Climate and agricultural practice interact to influence both crop production and soil microbes in agroecosystems. Here, we carried out a unique experiment in Central Germany to simultaneously investigate the effects of climates (ambient climate vs. future climate expected in 50-70 years), agricultural practices (conventional vs. organic farming), and their interaction on arbuscular mycorrhizal fungi (AMF) inside wheat (Triticum aestivum L.) roots. AMF communities were characterized using Illumina sequencing of 18S rRNA gene amplicons. We showed that climatic conditions and agricultural practices significantly altered total AMF community composition. Conventional farming significantly affected the AMF community and caused a decline in AMF richness. Factors shaping AMF community composition and richness at family level differed greatly among Glomeraceae, Gigasporaceae and Diversisporaceae. An interactive impact of climate and agricultural practices was detected in the community composition of Diversisporaceae. Organic farming mitigated the negative effect of future climate and promoted total AMF and Gigasporaceae richness. AMF richness was significantly linked with nutrient content of wheat grains under both agricultural practices. Lightening the injection burden is commonly believed to improve prophylaxis adherence. Efmoroctocog alfa (rFVIIIFc) is the first recombinant FVIII-Fc fusion protein available in France. This clotting factor with an extended half-life could thus improve medication adherence. The study primarily aimed to assess the real-life impact on prophylaxis adherence of haemophilia A patients, when switching from a standard to an extended half-life FVIII. This study was an observational, monocentre, non-interventional study aiming at assessing haemophilia A patients' real-life adherence during the first-year post-rFVIIIFc prophylaxis initiation. Medication adherence was assessed using two methods the medication possession ratio (MPR), which is based on the hospital pharmacy dispensing data, and self-reported VERITAS-Pro questionnaire. Patients on rFVIIIFc prophylaxis for at least 12months, following a 12-month standard FVIII prophylaxis, were eligible for inclusion. In 2019, 47 male patients were undergoing rFVIIIFc prophylaxis in our Hemophilia Center, among which 36 meeting the inclusion criteria.