Adequate cleavage of von Willebrand factor (VWF) prevents formation of thrombi. ADAMTS13 is the main VWF-cleaving protease and its deficiency results in development of thrombotic microangiopathy. Besides ADAMTS13 other proteases may also possess VWF-cleaving activity, but their physiological importance in preventing thrombus formation is unknown. This study investigated if, and which, proteases co

Complement is activated during Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS). There is evidence of complement activation via the alternative pathway in STEC-HUS patients as well as from in vivo and in vitro models. Ozaki et al. demonstrate activation of the mannose-binding lectin (MBL) pathway in Shiga toxin-treated mice expressing human MBL2, but lacking m

Enterohemorrhagic Escherichia coli (EHEC) is a highly pathogenic bacterial strain capable of causing watery or bloody diarrhea, the latter termed hemorrhagic colitis, and hemolytic-uremic syndrome (HUS). HUS is defined as the simultaneous development of non-immune hemolytic anemia, thrombocytopenia, and acute renal failure. The mechanism by which EHEC bacteria colonize and cause severe colitis, fo

The kidneys are the major organs affected in diarrhea-associated hemolytic uremic syndrome (D(+)HUS). The pathophysiology of renal disease in D(+)HUS is largely the result of the interaction between bacterial virulence factors such as Shiga toxin and lipopolysaccharide and host cells in the kidney and in the blood circulation. This chapter describes in detail the current knowledge of how these bac

Nonionic surfactants have broad applications such as cleaning and dispersion stabilization, which frequently are hampered by strong temperature sensitivities. As manifested by clouding and decreased solubility with increasing temperature, the interaction between water and the oligo(oxyethylene) head-groups is becoming less favorable. Different aspects of surfactant self-assembly, like the critical

PURPOSE: To examine the cytokine profile of epithelial cells lining the human urinary tract with the aim of differentiating between the constitutive and disease-related cytokine production in these tissues.MATERIALS AND METHODS: Sections from the renal pelvis, ureter, bladder or urethra were obtained from 22 patients undergoing urinary tract surgery and were stained with monoclonal antibodies to i

The plasma cryoprecipitate of two brothers with recurrent thrombotic thrombocytopenic purpura (TTP) was previously found to mediate increased platelet retention and contain ultra-large von Willebrand factor (vWF) multimers during remissions. We conducted this study to examine if vWF is involved in the increased platelet retention in TTP. Platelet retention decreased when the patients' plasma was i

In Bernard-Soulier syndrome, a hereditary bleeding disorder, the platelets are deficient in the glycoprotein (GP) Ib-IX-V complex, a major receptor for the von Willebrand factor. The components of the complex are encoded by separate genes. Patients with this syndrome have a variable expression level of the receptor protein on platelets depending on the specific genetic abnormality. We describe a p

The role of lipopolysaccharide (LPS) and Shiga-like toxin (SLT) in the pathogenesis of hemolytic uremic syndrome (HUS) was studied in a mouse model. Mice inoculated intragastrically with Escherichia coli O157:H7 developed gastrointestinal, neurologic, and systemic symptoms, necrotic foci in the colon, glomerular and tubular histopathology, and fragmented erythrocytes. LPS-responder (C3H/HeN) mice

In recent years, workflows within the field of contemporary classical music have changed drastically. Increasingly, composers are active in the process of creating and co-creating performances, not only the auditory dimensions but also the visual design and theatrical staging. The practice has recently been termed The New Discipline (Walshe, 2016). Of particular interest to the present article are

Diabetes is an increasing worldwide problem leading to major associated health issues and increased health care costs. In 2012, 9.3% of the American population was affected by diabetes, according to the American Diabetes Association, with 1.7 million of new cases since during the year (www.diabetes.org). Proteome initiatives can provide a deeper understanding of the biology of this disease and hel

Three-dimensional (3D) imaging of dynamic objects that rapidly undergoes structural changes, such as turbulent combusting flows, has been a long-standing challenge, mainly due to the common need for sequential image acquisitions. To accurately sense the 3D shape of the sample, all acquisitions need to be recorded within a sufficiently short time-scale during which the sample appears stationary. He

This paper studies the consequences on high temperature corrosion when biomass is co-fired with coal in oxy-fuel combustion, a concept that would enable negative CO2 emissions by combining renewable fuels with CO2 capture. The particular focus of the work is the sulphation of alkali chlorides which is studied by means of experiments and modelling. The melting point of alkali sulphates is higher th

Irradiating a flame by microwave radiation is one of several plasma-assisted combustion (PAC) technologies that can be used to modify the combustion chemical kinetics in order to improve flame-stability and to delay lean blow-out. One practical implication is that engines may be able to operate with leaner fuel mixtures and have an improved fuel flexibility capability including biofuels. In additi

Single-shot imaging of methyl radical (CH3) in premixed methane/air flames is demonstrated using photofragmentation laser-induced fluorescence (PF-LIF) technique. A pump-probe strategy was adopted with the pump laser at 212.8 nm photolyzing CH3, and with the probe laser at 426.8 nm detecting the photolyzed CH (X 2Π) fragments. Spatially resolved spectrograph of the PF-LIF signal from a stable lami

Flame emission spectra and OH* imaging are carried out together with planar laser-induced fluorescence (PLIF) of OH and CH2O to characterize the reaction zone structures of three combustion modes, i.e. a flame mode, a transition mode and a flameless mode, established in a laboratory combustor. Spectroscopic measurements indicate that the invisibility of the flameless mode can be attributed to a mo