OBJECTIVE: Diamond-Blackfan anemia (DBA) is a rare congenital hypoplastic anemia caused by mutations in ribosomal protein (RP) genes. Our aim is to develop gene therapy for DBA patients with mutations in RPS19. We previously demonstrated that RPS19 gene transfer partially corrects erythroid development in vitro. In this study, we asked if RPS19 gene transfer corrects erythroid development in unsor

Objective:To examine the impact of non-response to self-reported body weight and height in health questionnaires for the estimation of obesity prevalence.Methods and Procedures:A cross-sectional population-based health survey in the community ofVara with 16,009 residents (in year 2002) in South-western Sweden. Participants randomly selected in strata by sex and age among residents being 30-74 year

Multipath fading is one of the main challenges in transmission over wireless broadband relay channels due to frequency selectivity, which may deteriorate the received signal. Exploiting the extra source of multipath diversity, aside from cooperative (user) diversity, is important when coping with these wireless channel limitations. In this correspondence, we propose a new distributed space-frequen

Clonogenic neural stem (NS) cell lines grown in adherent cultures have previously been established from embryonic stem cells and fetal and adult CNS in rodents and from human fetal brain and spinal cord. Here we describe the isolation of a new cell line from human fetal striatum (hNS cells). These cells showed properties of NS cells in vitro such as monolayer growth, high proliferation rate and ex

Lymphocytic infiltration of different exocrine and non-exocrine epithelia is the pathological hallmark of primary Sjogren's syndrome, whereas involvement of salivary and lachrymal glands with the clinical counterpart of dry eye and dry mouth are the predominant features of the disease, together with fatigue and musculoskeletal pain. In addition, systemic manifestations, like arthritis, skin vascul

In recent years, the Galactic Centre (GC) region (200 pc in radius) has been studied in detail with spectroscopic stellar data as well as an estimate of the ongoing star formation rate. The aims of this paper are to study the chemical evolution of the GC region by means of a detailed chemical evolution model and to compare the results with high-resolution spectroscopic data in order to impose cons

The aim of this study was to assess cardiovascular predictors for all-cause long-term mortality in patients undergoing standard endovascular aneurysm repair (EVAR) for infrarenal abdominal aortic aneurysm (AAA). Consecutive patients treated with EVAR (Zenith (R) stent grafts; Cook) between May 1998 and February 2006 were prospectively enrolled in a computerized database, together with retrospectiv

Desmoplastic fibroma (DFB) is a benign primary bone tumor that usually occurs in adolescents and young adults. The genetic information on DFB is very limited. We here present cytogenetic, fluorescence in situ hybridization and single nucleotide polymorphism array findings in a case that had a rearrangement involving chromosomes 11 and 19 at G-banding analysis. The results showed that the breakpoin

We use a recently developed continuum theory to expand on an exact treatment of the interfacial properties of telechelic polymers displaying Schulz-Flory polydispersity. Our results are remarkably compact and can be derived from the properties of equilibrium, ideal polymers at interfaces. A new surface adsorption transition is identified for ideal telechelic chains, wherein the central block is an

To investigate which factors and criteria are used in priority setting of pharmaceuticals, in what contexts health economic evaluations are used, and barriers to the use of health economic evaluations at micro, meso, and macro health-care levels.

The aim of this paper is to explore the complexity of the concept of the victim within the context of organ trading. By examining the intricate phenomenon of organ trade, we show how prevailing notions of victimhood form the basis of concrete social practices. The empirical basis for this exploration comprises in-depth interviews conducted during fieldwork in South Africa and Kosovo. We also draw

Wilms tumor (WT) is a pediatric tumor of the kidney, the treatment of which includes heavy chemotherapy. Affected children would likely benefit from more targeted therapies with limited side effects. Establishment of relevant orthotopic WT xenografts is important to better understand mechanisms of WT growth and for preclinical drug testing.

GLP-1 receptor agonists may provide an alternative to prandial insulin for advancing basal insulin therapy. Harmony 6 was a randomized, open-label, active-controlled trial testing once-weekly albiglutide vs thrice-daily prandial insulin lispro as an add-on to titrated once-daily insulin glargine.RESEARCH DESIGN AND METHODS: Patients taking basal insulin (with or without oral agents) with HbA1c 7-1

To evaluate the expression profiles of cell-free plasma miRNAs in SSc and to characterize their correlation with disease subgroups (lcSSc and dcSSc) and with autoantibody profiles.

A role for the immune system in the pathogenesis of Parkinson's Disease (PD) has previously been suggested. A recent genome-wide association (GWA) study identified an association between one single nucleotide polymorphism (SNP) in the human leucocyte antigen (HLA) region (HLA-DRA rs3129882) and PD in a population of American patients with European ancestry. In that study, the minor rs3129882 allel

The effective mechanical properties of polymethyl-methacrylate (PMMA) reinforced with carbon nanotubes (CNTs) were evaluated by means of two approaches: experiments and a micromechanical model. With various concentrations of CNTs, two specimen fabrication processes were examined: hot pressing (HP) and injection molding (IM). Experiments included a series of uniaxial tensile tests guided by an ASTM

Context:Genome-wide association studies (GWASs) of differentiated thyroid cancer (DTC) have identified associations with polymorphisms at 2q35 (DIRC3), 8p12 (NRG1), 9q22.33 (FOXE1) and 14q13.2 (NKX2-1). However, most of the inherited genetic risk factors of DTC remain to be discovered.Objective:Our objective was to identify additional common DTC susceptibility loci.Design:We conducted a GWAS in a

Background Multiple myeloma cells uniformly overexpress CD38. We studied daratumumab, a CD38-targeting, human IgG1κ monoclonal antibody, in a phase 1-2 trial involving patients with relapsed myeloma or relapsed myeloma that was refractory to two or more prior lines of therapy. Methods In part 1, the dose-escalation phase, we administered daratumumab at doses of 0.005 to 24 mg per kilogram of body

Epitaxial graphene growth was performed on the Si-terminated face of 4H-, 6H-, and 3C-SiC substrates by silicon sublimation from SiC in argon atmosphere at a temperature of 2000 degrees C. Graphene surface morphology, thickness and band structure have been assessed by using atomic force microscopy, low-energy electron microscopy, and angle-resolved photoemission spectroscopy, respectively. Differe