Clonal B-cell proliferation is a frequent manifestation of Gaucher disease-a sphingolipidosis associated with a high risk of multiple myeloma and non-Hodgkin lymphoma. Gaucher disease is caused by genetic deficiency of acid -glucosidase, the natural substrates of which (-d-glucosylceramide and -d-glucosylsphingosine) accumulate, principally in macrophages. Mice with inducible deficiency of -glucos

In systemic sclerosis (SSc)-related interstitial lung disease (ILD), elevated eosinophil counts in bronchoalveolar lavage are associated with a worse outcome. We hypothesized that eosinophils may be activated in the peripheral circulation, thereby increasing their recruitment to affected tissues and contributing to inflammation and fibrosis. The aim of this study was to characterize the blood eosi

Background: Exposure to inhaled endotoxins (lipopolysaccharides, LPS) of Gram-negative bacteria commonly found in indoor environments and assessed in secondary tobacco smoke, has been associated with airway inflammation and asthma exacerbation. The bronchoalveolar lavage fluid (BALf) from patients with interstitial lung diseases (sarcoidosis, lung fibrosis, smoking-related ILD, eosinophilic disord

Purpose The (14;18) translocation constitutes both a genetic hallmark and critical early event in the natural history of follicular lymphoma (FL). However, t(14;18) is also detectable in the blood of otherwise healthy persons, and its relationship with progression to disease remains unclear. Here we sought to determine whether t(14;18)-positive cells in healthy individuals represent tumor precurso

The effect of deglycosylation of pyranose dehydrogenase (PDH), obtained from Agaricus meleagris and recombinantly expressed in Pichia pastoris, on the amperometric detection of glucose was investigated. Glycosylated (gPDH) and deglycosylated (dgPDH) PDH were immobilized on spectrographic graphite (G) simultaneously with an Os redox polymer (Os-RP). The amperometric response of G/Os-RP/gPDH and G/O

Background:CLL is a chronic disease that often progresses slowly from a precursor stage, monoclonal B-cell lymphocytosis (MBL), and that can remain undiagnosed for a long time. Methods:Within the European EPIC cohort, we measured pre-diagnostic plasma sCD23 for 179 individuals who eventually were diagnosed with CLL and an equal number of matched control subjects who remained free of cancer. Result

The present study examined mRNA levels of apolipoprotein M (apoM) and apolipoprotein AI (apoAI) in the term placental tissues obtained from 37 women with normal birth weight neonates and from 37 women with macrosomic neonates (birth body weight a parts per thousand yen4,000 g), and further discussed possible clinical significance of these observations. The mRNA levels of apoM and apoAI in the plac

Purpose To ascertain the clinical implications of high hyperdiploid (HH; 49-65 chromosomes) and tri-/tetraploid (TT; >65 chromosomes) adult acute myeloid leukemia (AML), all such cases were retrieved from the Swedish AML Registry. Results Of the 3,654 cytogenetically informative cases diagnosed between January 1997 and May 2014, 68 (1.9%) were HH (n=50)/TT (n=18). Patients with HH/TT were older th

While good physiological models of the glucose metabolism in type 1 diabetic patients are well known, their parameterisation is difficult. The high intra-patient variability observed is a further major obstacle. This holds for data-based models too, so that no good patient-specific models are available. Against this background, this paper proposes the use of interval models to cover the different

Background.The currently used patch test concentration for formaldehyde is 1.0% (wt/vol) in water. However, clinical experience and previous studies suggest that 1.0% might be insufficient for detecting an optimized number of clinically relevant cases of contact allergy to formaldehyde. Objectives.To validate earlier patch test results for comparison of 1% (wt/vol) and 2% (wt/vol) formaldehyde in

The Medical ANtiarrhythmic Treatment or Radiofrequency Ablation in Paroxysmal Atrial Fibrillation (MANTRA-PAF) trial assessed the long-term efficacy of an initial strategy of radiofrequency ablation (RFA) vs. antiarrhythmic drug therapy (AAD) as first-line treatment for patients with PAF. In this substudy, we evaluated the effect of these treatment modalities on the Health-Related Quality of Life

The Src-Like Adaptor Protein (SLAP) is an adaptor protein sharing considerable structural homology with Src. SLAP is expressed in variety of cells regulating receptor tyrosine kinase signaling by direct association. In this report, we show that SLAP associates with both wild-type and oncogenic c-Kit (c-Kit-D816V). The association involves SLAP SH2 domain and receptor phosphotyrosine residues diffe

This paper examines whether a resident's estimate of five traffic-related phenomena (accident frequency, incident frequency, difficulty of crossing the street, traffic flow, and speed level) in their neighborhood may depend on factors such as gender, age, how frequently they walk along the street or on characteristics of the street. The material consists of survey data from 919 residents living al

3-Hydroxypropionic acid (3-HP), an important C3 chemical for a bio-based industry, is natively produced by Lactobacillus reuteri from glycerol. Conversion of glycerol occurs via the intermediate 3-hydroxypropionaldehyde (3-HPA), followed by an ATP-producing pathway initiated by the CoA-acylating propionaldehyde dehydrogenase (PduP). The pduP gene of L. reuteri was cloned and expressed in Escherich

The extent to which a system of services is in tune with the needs of the population can be expressed in terms of target efficiency, which includes horizontal target efficiency - the extent to which those deemed to need a service receive it - and vertical target efficiency - the corresponding extent to which those who receive a service actually need it. Vertical efficiency can be measured by looki

Meltwater beneath the large ice sheets can influence ice flow by lubrication at the base or by softening when meltwater refreezes to form relatively warm ice. Refreezing has produced large basal ice units in East Antarctica. Bubble-free basal ice units also outcrop at the edge of the Greenland ice sheet5, but the extent of refreezing and its influence on Greenland’s ice flow dynamics are unknown.

Based on constructal theory, five different cases with multistage bifurcations are designed as well as one case without bifurcations, and the corresponding laminar fluid flow and thermal performance have been investigated numerically. All laminar fluid flow and heat transfer results are obtained using computation fluid dynamics, and a uniform wall heat flux thermal boundary condition is applied al

The upgraded tagged photon facility at the MAX IV Laboratory is presented. The pulsed electron beam from a linac system is stretched in the MAX I ring and the electron beam hits a thin aluminium foil in which bremsstrahlung is produced. One of two available magnetic spectrometers is used to determine the energy of the post-bremsstrahlung electrons. The tagged photon ranges from 10 to 180 MeV with