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Objective: The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). Subjects and methods: To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and ano

Popular Abstract in Swedish Blodförgiftningen (sepsis) kan ge upphov till akut hjärtsvikt, vilken på cellulär nivå karakteriseras av minskad känslighet för kalciumjonen och de vanliga hjärtförstärkande medicinerna (inotropa farmaka). Avhandlingen handlar om tidig behandling av sepsis-associerad hjärtsvikt med den kalciumsensitiseraren levosimendan, ett nytt läkemedel som höjer hjärtats kontraktionSepsis-related cardiovascular dysfunction associated with fluid-unresponsive tissue hypoperfusion might require inotropic treatment. This cardiovascular dysfunction seems to involve calcium desensitization and adrenergic unresponsiveness. We investigated the effects of clinically relevant plasma concentrations of the calcium sensitizer levosimendan during the first 6 h of endotoxemia in a model of

Background Transcatheter aortic valve replacement (TAVR) has become an established therapeutic option for patients with symptomatic, severe aortic valve stenosis (AS) who are ineligible or at high risk for conventional valvular surgery. In Northwestern Europe, the TAVR technology is also increasingly used to treat patients with an intermediate risk profile. Methods and results The study was design

Elevated manganese (Mn) in drinking water has been reported worldwide. While, naturally occurring Mn in groundwater is generally the major source, anthropogenic contamination by Mn-containing fungicides such as mancozeb may also occur. The main objective of this study was to examine factors associated with Mn and ethylenethiourea (ETU), a degradation product of mancozeb, in drinking water samples

Forest fires are an important disturbance factor of boreal forests, annually burning about 0.5% of the forested area in Canada. Wildfire regimes are influenced by climate and a number of studies project an increase in wildfire activity with climate change. Another factor influencing wildfires is human intervention (fire suppression), and one factor that has rarely been assessed is fuel fragmentati

This paper discusses the design and analysis of a reflector antenna system combining two doubly-curved Circular Polarization Selective Surfaces to provide a functionality equivalent to that of a dual gridded reflector but in circular polarization. The key feature of this design is to use a non-resonant CPSS design to avoid the bandwidth limitation of most CPSS designs previously discussed in the l

Prior treatment exposure in patients with relapsed/refractory multiple myeloma may affect outcomes with subsequent therapies. We analyzed efficacy and safety according to prior treatment in the phase 3 TOURMALINE-MM1 study of ixazomib-lenalidomide-dexamethasone (ixazomib-Rd) versus placebo-Rd. Patients with relapsed/refractory multiple myeloma received ixazomib-Rd or placebo-Rd. Efficacy and safet

Tau neuronal and glial pathologies drive the clinical presentation of Alzheimer's disease and related human tauopathies. There is a growing body of evidence indicating that pathological tau species can travel from cell to cell and spread the pathology through the brain. Throughout the last decade, physiological and pathological tau have become attractive targets for AD therapies. Several therapeut

G protein-coupled receptor 30 (GPR30) is a G protein-coupled receptor (GPCR) that is attracting considerable attention in breast cancer and cardiometabolic regulation. Following reports that GPR30 is required for some rapid estrogen responses, e.g. increased cAMP production and ERK1/2 activity, in estrogen receptor (ER)-negative cells, GPR30 was renamed G protein-coupled estrogen receptor 1 (GPER1

Atopic dermatitis (AD) is a clinically defined, highly pruritic, chronic inflammatory skin disease of children and adults. The diagnosis is made using evaluated clinical criteria. Disease activity is best measured with a composite score assessing both objective signs and subjective symptoms, such as SCORAD. The management of AD must consider the clinical and pathogenic variabilities of the disease

GPR30, or G protein-coupled estrogen receptor (GPER), is a G protein-coupled receptor (GPCR) that is currently attracting considerable attention in breast cancer and cardiometabolic regulation. The receptor was reported to be a novel membrane estrogen receptor mediating rapid non-genomic responses. However, questions remain about both the cognate ligand and the subcellular localization of receptor

Beside its classical role as a serum effector system of innate immunity, evidence is accumulating that complement has an intracellular repertoire of components that provides not only immune defense, but also functions to maintain cellular homeostasis. While complement proteins, mainly the central component C3, have been detected in B cells, their exact function and source remain largely unexplored

Background: Many inborn errors of metabolism (IEMs) may present as sudden infant death (SID). Nowadays, increasing numbers of patients with IEMs are identified pre-symptomatically by population neonatal bloodspot screening (NBS) programmes. However, some patients escape early detection because their symptoms and signs start before NBS test results become available, they even die even before the sa

We present the results of dynamic in-situ 3D X-ray imaging of methane hydrates microstructure during methane hydrate formation and dissociation in sand samples. Short scanning times and high resolution provided by synchrotron X-rays allowed for better understanding of water movement and different types of gas-hydrate formation. Complementing previous observations, we conclude that the process of g

AIMS: The CHA2DS2VASc score is used to evaluate the risk of thromboembolic events in patients with non-valvular atrial fibrillation. We assessed the prognostic yield of CHA2DS2VASc for new-onset atrial fibrillation, cardiovascular morbidity and mortality in a non-atrial fibrillation population.METHODS: We analysed a population-based cohort of 22,179 middle-aged individuals with (n = 3542) and with

A fundamental challenge in medical microbiology is to characterize the dynamic protein-protein interaction networks formed at the host-pathogen interface. Here, we generate a quantitative interaction map between the significant human pathogen, Streptococcus pyogenes, and proteins from human saliva and plasma obtained via complementary affinity-purification and bacterial-surface centered enrichment

Introduction: Previous studies have shown that the injection of adipose tissue–derived stem cells (ADSCs) into the tunica albuginea (TA) during the active phase of Peyronie's disease (PD) prevents the development of fibrosis. Aim: To investigate, using an animal model, whether local injection of human ADSCs (hADSCs) can alter the degree of fibrosis in the chronic phase of PD. Methods: 27 male, 12-