Efficacy of Wilate Prophylaxis in Reducing Nosebleeds in Patients with Severe VWD - A Post-hoc Analysis of the WIL-31 Study.

Background: Prophylaxis with a von Willebrand factor (VWF) concentrate is recommended in von Willebrand disease (VWD) patients with a history of frequent and severe bleeds. Despite nosebleeds being a frequent manifestation of VWD, few studies have investigated the efficacy of factor prophylaxis in preventing nosebleeds in patients with severe VWD.

Methods: This post-hoc analysis of a prospective, 12-month, phase 3 study assessed the efficacy of wilate in the prevention of nosebleeds in 33 patients aged ≥6 years with severe type 1, type 2 or type 3 VWD.

Binding of a Tricationic meso-Substituted Porphyrin to poly(A)⋅poly(U): an Experimental Study.

The porphyrins are macrocyclic compounds widely used as photosensitizers in anticancer photodynamic therapy. The binding of a tricationic meso-tris(N-methylpyridinium)-porphyrin, TMPyP, to poly(A)⋅poly(U) polynucleotide has been studied in neutral buffered solution, pH6.9, of low and near-physiological ionic strength in a wide range of molar phosphate-to-dye ratios (P/D).

Stromal cell and B cell dialogue potentiates IL-33-enriched lymphoid niches to support eosinophil recruitment and function during type 2 immunity.

Eosinophils are involved in host protection against multicellular organisms. However, their recruitment to the mesenteric lymph node (mLN) during type 2 immunity is understudied. Our results demonstrate that eosinophil association with lymphoid stromal niches constructed by fibroblastic reticular cells (FRCs) and lymphatic endothelial cells is diminished in mice selectively lacking interleukin (IL)-4Rα or lymphotoxin-β (LTβ) expression on B cells.

Facile Synthesis of a Crystalline Zinc Sulfide/Chitosan Biopolymer Nanocomposite: Characterization and Application for Photocatalytic Degradation of Textile Dyes and Anticancer Activity.

In the present study, we have synthesized a zinc sulfide/chitosan (ZS/CS) nanocomposite by utilizing simple, economical, and environmentally friendly methods. The synthesized nanomaterials were characterized by different analytical techniques such as XRD, FE-SEM, EDS, and FTIR to determine the phase structure, morphology, and elemental composition. FTIR spectroscopy was used to confirm the functional groups of the synthesized zinc sulfide (ZS) nanoparticles and ZS/CS composite.

von Willebrand factor/factor VIII concentrate (Wilate) prophylaxis in children and adults with von Willebrand disease.

Long-term prophylaxis with a von Willebrand factor (VWF) concentrate is recommended in patients with von Willebrand disease (VWD) who have a history of severe and frequent bleeds. However, data from prospective studies are scarce. WIL-31, a prospective, noncontrolled, international phase 3 trial, investigated the efficacy and safety of Wilate prophylaxis in severe patients with VWD.

Simoctocog alfa (Nuwiq®) in previously untreated patients with severe haemophilia A-Final efficacy and safety results from the NuProtect study.

Introduction: Simoctocog alfa (Nuwiq®) is a 4th generation recombinant FVIII with proven efficacy for the prevention and treatment of bleeding episodes (BEs) in previously treated patients with severe haemophilia A. The NuProtect study assessed the immunogenicity, efficacy and safety of simoctocog alfa in 108 previously untreated patients (PUPs). The incidence of high-titre inhibitors was 16.

Long-term immunogenicity, efficacy and tolerability of simoctocog alfa in patients with severe haemophilia A who had completed the NuProtect study in previously untreated patients.

Background: The NuProtect study reported data on the immunogenicity, efficacy and tolerability of simoctocog alfa (Nuwiq ) in 108 previously untreated patients with severe haemophilia A planned to be treated for ≥100 exposure days or up to 5 years. The NuProtect-Extension study collected long-term prophylaxis data in children with severe haemophilia A.

Methods: Patients who completed the NuProtect study according to the protocol were eligible for the NuProtect-Extension study, a prospective, multinational, non-controlled, Phase 3b study.

An enhanced fluorescent sensor system based on molecularly imprinted polymer chips with silver nanoparticles for highly-sensitive zearalenone analysis.

A novel enhanced fluorescent sensor system for zearalenone (ZON) determination in flour samples is presented. The ZON-selective molecularly imprinted polymer (MIP) films were developed with a computational modelling method and synthesised with cyclododecyl-2,4-dihydroxybenzoate as a "dummy" template and ethylene glycol methacrylate phosphate as a functional monomer acted as the selective recognition elements for ZON fluorescence detection. Spherical silver nanoparticles (AgNPs) were embedded in the MIP films' structure to enhance the sensor sensitivity.

A Prospective Observational Study to Evaluate the Factors Affecting the Dermatology Life Quality Index in Patients With Hidradenitis Suppurativa.

Background: Hidradenitis suppurativa (HS) is a chronic, relapsing inflammatory skin disease that significantly affects the quality of life of patients. Multiple factors affect the disease's course and severity. HS is a debilitating disease and often recalcitrant to treatment, resulting in a deterioration of quality of life; hence, there is a need to evaluate the factors affecting the quality of life in patients with HS.

Minimum detection limits of the TROPOMI satellite sensor across North America and their implications for measuring oil and gas methane emissions.

Methane emissions from natural gas are of ever-increasing importance as we struggle to reach Paris climate targets. Locating and measuring emissions from natural gas can be particularly difficult as they are often widely distributed across supply chains. Satellites are increasingly used to measure these emissions, with some such as TROPOMI giving daily coverage worldwide, making locating and quantifying these emissions easier.

COVID-19-induced coagulopathy: Experience, achievements, prospects.

The presence of coagulopathy as part of the systemic inflammatory response syndrome is a characteristic feature of severe coronavirus disease 2019 (COVID-19). Hematological changes (increased D-dimer [DD], prolonged activated partial thromboplastin clotting time [APTT] and prothrombin time [PT], high fibrinogen levels) have been observed in hospitalized patients with COVID-19, which characterize the risk of thrombotic events. Against the background of COVID-19 there is endothelial dysfunction, hypoxia and pulmonary congestion, mediated by thrombosis and microvascular occlusion.

A Randomized Comparative Study of Intralesional Tranexemic Acid and Kligman's Regimen in Indian Patients with Melasma.

Context: Melasma is an acquired chronic disorder of hyperpigmentation. Tranexamic acid (TXA) has been shown to be effective in reducing the severity of melasma.

Aims: The aim of this study is to compare the efficacy of intralesional TXA with topical Kligman's regimen in the treatment of facial melasma and to assess their safety profile.

Hydrogen emissions from the hydrogen value chain-emissions profile and impact to global warming.

Future energy systems could rely on hydrogen (H) to achieve decarbonisation and net-zero goals. In a similar energy landscape to natural gas, H emissions occur along the supply chain. It has been studied how current gas infrastructure can support H, but there is little known about how H emissions affect global warming as an indirect greenhouse gas.

Unfriendly Fire: How the Tobacco Industry is Destroying the Future of Our Children.

Tobacco has long been known to be one of the greatest causes of morbidity and mortality in the adults, but the effects on the foetus and young children, which are lifelong, have been less well appreciated. Developing from this are electronic nicotine delivery systems or vapes, promulgated as being less harmful than tobacco. Nicotine itself is toxic to the foetus, with permanent effects on lung structure and function.

Simoctocog Alfa (Nuwiq) in Previously Untreated Patients with Severe Haemophilia A: Final Results of the NuProtect Study.

Introduction:  FVIII inhibitor development is the most serious contemporary treatment complication in haemophilia A, particularly in previously untreated patients (PUPs). No inhibitors developed in clinical trials in previously treated patients treated with simoctocog alfa (Nuwiq), a fourth-generation recombinant FVIII produced in a human cell line.

Methods:  The NuProtect study investigated the immunogenicity of simoctocog alfa in PUPs.

Sensor Based on Molecularly Imprinted Polymer Membranes and Smartphone for Detection of Contamination in Cereals.

The combination of the generic mobile technology and inherent stability, versatility and cost-effectiveness of the synthetic receptors allows producing optical sensors for potentially any analyte of interest, and, therefore, to qualify as a platform technology for a fast routine analysis of a large number of contaminated samples. To support this statement, we present here a novel miniature sensor based on a combination of molecularly imprinted polymer (MIP) membranes and a smartphone, which could be used for the point-of-care detection of an important food contaminant, oestrogen-like toxin zearalenone associated with contamination of cereals. The detection is based on registration of natural fluorescence of zearalenone using a digital smartphone camera after it binds to the sensor recognition element.

IL-4Rα-Expressing B Cells Are Required for CXCL13 Production by Fibroblastic Reticular Cells.

Adaptive type 2 immune responses against the intestinal helminth Heligmosomoides polygyrus (Hp) require the interaction of follicle-associated CXCR5 dendritic cells with naive T cells in the draining mesenteric lymph nodes (mLNs). However, the source of CXCL13 responsible for attracting CXCR5 dendritic cells has remained unclear. Using multiplex imaging combined with deep tissue analysis, we observed new CXCL13 fibroblastic reticular cells surrounding paracortical and cortical B cell follicles in the mLNs of infected mice.

Development of Sample-Adaptable Holders for Lightsheet Microscopy.

Multi-user core microscopy facilities are often faced with the challenge to adapt or modify existing instruments. This is essential in order to fulfill the requirements of the user community, who wants to image a wide range of model organisms with varying stains and sample thicknesses. In recent years, lightsheet microscopy has turned into an invaluable tool for both live and cleared sample imaging of many different specimens.

Vilnius Declaration on chronic respiratory diseases: multisectoral care pathways embedding guided self-management, mHealth and air pollution in chronic respiratory diseases.

Background: Over 1 billion people suffer from chronic respiratory diseases such as asthma, COPD, rhinitis and rhinosinusitis. They cause an enormous burden and are considered as major non-communicable diseases. Many patients are still uncontrolled and the cost of inaction is unacceptable.

FIBCD1 Binds and Regulates Lung Epithelial Response to Cell Wall Components.

() is a ubiquitous fungus of clinical importance associated with development of various pulmonary diseases and allergic hypersensitivity reactions. It is protected against environmental stress by a cell wall that contains polysaccharides such as chitin. We previously demonstrated that fibrinogen C domain-containing protein 1 (FIBCD1) is a membrane-bound protein that binds chitin through a conserved S1 binding site and is expressed in intestinal epithelium and salivary glands.

Ruptured Sinus of Valsalva: An Unusual Cause of Heart Failure.

A 35-year-old male patient presented with heart failure. On examination, there was a continuous murmur over the left sternum. Transthoracic echocardiography revealed a ruptured aneurysm arising from the noncoronary sinus of Valsalva and draining into the right atrium.