Differences in weight status and autonomous motivation towards sports among children with various profiles of motor competence and organized sports participation

This study aimed (1) to identify profiles in children based on actual motor competence (AMC), perceived motor competence (PMC), and organized sports participation (OSP), and (2) to examine differences among these profiles in weight status as well as autonomous motivation towards sports. Children's (N = 206; 112 boys; Mage = 10.83 ± 0.92 years) AMC, PMC, OSP, weight status, and autonomous motivation towards sports were measured using validated assessment tools. Cluster analyses identified three profiles with completely convergent levels of AMC, PMC, and OSP and three profiles with partially convergent levels. Children in the convergent profiles with average to high levels of AMC, PMC, and OSP had the most optimal profile, as they combined a healthier weight status with elevated levels of autonomous motivation, while the opposite was true for children with low levels on all three cluster-variables. Partially convergent profiles showed that AMC and PMC appear crucial for weight status, as profiles with relatively low levels of AMC and PMC had the highest weight status, independent of their OSP levels. Overall, the findings highlight the importance of promoting AMC, PMC, and OSP simultaneously to help children in achieving a healthy weight status and being autonomously motivated towards OSP

Approximate conditional distributions of distances between nodes in a two-dimensional sensor network

When we represent a network of sensors in Euclidean space by a graph, there are two distances between any two nodes that we may consider. One of them is the Euclidean distance. The other is the distance between the two nodes in the graph, defined to be the number of edges on a shortest path between them. In this paper, we consider a network of sensors placed uniformly at random in a two-dimensional region and study two conditional distributions related to these distances. The first is the probability distribution of distances in the graph, conditioned on Euclidean distances; the other is the probability density function associated with Euclidean distances, conditioned on distances in the graph. We study these distributions both analytically (when feasible) and by means of simulations. To the best of our knowledge, our results constitute the first of their kind and open up the possibility of discovering improved solutions to certain sensor-network problems, as for example sensor localization

Model-consistent estimation of the basic reproduction number from the incidence of an emerging infection

We investigate the merit of deriving an estimate of the basic reproduction number \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document} $$\mathcal{R}_0$$ \end{document} early in an outbreak of an (emerging) infection from estimates of the incidence and generation interval only. We compare such estimates of \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document} $$\mathcal{R}_0$$ \end{document} with estimates incorporating additional model assumptions, and determine the circumstances under which the different estimates are consistent. We show that one has to be careful when using observed exponential growth rates to derive an estimate of \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document} $$\mathcal{R}_0$$ \end{document} , and we quantify the discrepancies that arise

Heterozygous Loss-of-Function Mutations in DLL4 Cause Adams-Oliver Syndrome.

Adams-Oliver syndrome (AOS) is a rare developmental disorder characterized by the presence of aplasia cutis congenita (ACC) of the scalp vertex and terminal limb-reduction defects. Cardiovascular anomalies are also frequently observed. Mutations in five genes have been identified as a cause for AOS prior to this report. Mutations in EOGT and DOCK6 cause autosomal-recessive AOS, whereas mutations in ARHGAP31, RBPJ, and NOTCH1 lead to autosomal-dominant AOS. Because RBPJ, NOTCH1, and EOGT are involved in NOTCH signaling, we hypothesized that mutations in other genes involved in this pathway might also be implicated in AOS pathogenesis. Using a candidate-gene-based approach, we prioritized DLL4, a critical NOTCH ligand, due to its essential role in vascular development in the context of cardiovascular features in AOS-affected individuals. Targeted resequencing of the DLL4 gene with a custom enrichment panel in 89 independent families resulted in the identification of seven mutations. A defect in DLL4 was also detected in two families via whole-exome or genome sequencing. In total, nine heterozygous mutations in DLL4 were identified, including two nonsense and seven missense variants, the latter encompassing four mutations that replace or create cysteine residues, which are most likely critical for maintaining structural integrity of the protein. Affected individuals with DLL4 mutations present with variable clinical expression with no emerging genotype-phenotype correlations. Our findings demonstrate that DLL4 mutations are an additional cause of autosomal-dominant AOS or isolated ACC and provide further evidence for a key role of NOTCH signaling in the etiology of this disorder

Structural genomic variants in thoracic aortic disease.

PURPOSE OF REVIEW: Structural genomic variants have emerged as a relevant cause for several disorders, including intellectual disability, neuropsychiatric disorders, cancer and congenital heart disease. In this review, we will discuss the current knowledge about the involvement of structural genomic variants and, in particular, copy number variants in the development of thoracic aortic and aortic valve disease. RECENT FINDINGS: There is a growing interest in the identification of structural variants in aortopathy. Copy number variants identified in thoracic aortic aneurysms and dissections, bicuspid aortic valve related aortopathy, Williams-Beuren syndrome and Turner syndrome are discussed in detail. Most recently, the first inversion disrupting FBN1 has been reported as a cause for Marfan syndrome. SUMMARY: During the past 15 years, the knowledge on the role of copy number variants as a cause for aortopathy has grown significantly, which is partially due to the development of novel technologies including next-generation sequencing. Although copy number variants are now often investigated on a routine basis in diagnostic laboratories, more complex structural variants such as inversions, which require the use of whole genome sequencing, are still relatively new to the field of thoracic aortic and aortic valve disease

Infection dynamics and persistence of hepatitis E virus on pig farms – a review

Background: Hepatitis E virus (HEV) genotype 3 and 4 is a zoonosis that causes hepatitis in humans. Humans can become infected by consumption of pork or contact with pigs. Pigs are the main reservoir of the virus worldwide and the virus is present on most pig farms. Main body: Though HEV is present on most farms, the proportion of infected pigs at slaughter and thus the level of exposure to consumers differs between farms and countries. Understanding the cause of that difference is necessary to install effective measures to lower HEV in pigs at slaughter. Here, HEV studies are reviewed that include infection dynamics of HEV in pigs and on farms, risk factors for HEV farm prevalence, and that describe mechanisms and sources that could generate persistence on farms. Most pigs become infected after maternal immunity has waned, at the end of the nursing or beginning of the fattening phase. Risk factors increasing the likelihood of a high farm prevalence or proportion of actively infected slaughter pigs comprise of factors such as farm demographics, internal and external biosecurity and immunomodulating coinfections. On-farm persistence of HEV is plausible, because of a high transmission rate and a constant influx of susceptible pigs. Environmental sources of HEV that enhance persistence are contaminated manure storages, water and fomites. Conclusion: As HEV is persistently present on most pig farms, current risk mitigation should focus on lowering transmission within farms, especially between farm compartments. Yet, one should be aware of the paradox of increasing the proportion of actively infected pigs at slaughter by reducing transmission insufficiently. Vaccination of pigs may aid HEV control in the future.</p

IPSC reprogramming of two patients with spondyloepimetaphyseal dysplasia (SEMD, biglycan type).

Hemizygous missense variants in the X-linked BGN gene, encoding the extracellular matrix protein biglycan, cause spondyloepimetaphyseal dysplasia (SEMD, biglycan type), which is clinically characterized by short stature, brachydactyly and osteoarthritis. Little is known about the pathomechanisms underlying SEMD, biglycan type. IPSC-derived chondrocyte disease models have been shown to exhibit several key aspects of known disease mechanisms of skeletal dysplasias and are therefore considered highly suitable human disease models to study SEMD, biglycan type. Prior to creating iPSC-chondrocytes, dermal fibroblasts of two male patients with SEMD, biglycan type, carrying the p.Gly259Val variant were successfully reprogrammed into iPSCs using the CytoTune(TM)-iPS 2.0 Sendai Kit (Invitrogen)

Effects of dietary arabinoxylan-oligosaccharides (AXOS) and endogenous probiotics on the growth performance, non-specific immunity and gut micrbiota of juvenile Siberian sturgeon (Acipenser baerii)

We investigated the effects of administration of putative endogenous probiotics Lactococcus lactis spp. lactis or Bacillus circulans, alone and in combination with arabinoxylan-oligosaccharides (AXOS), a new class of candidate prebiotics, in juvenile Siberian sturgeon (Acipenser baerii). Eight experimental diets were tested: basal diet (Diet 1), basal diet supplemented with 2% AXOS (Diet 2), or L. lactis ST G81 (Diet 3), L. lactis ST G45 (Diet 4), B. circulans ST M53 (Diet 5), L. lactis ST G81 + 2% AXOS (Diet 6), L. lactis ST G45 + 2% AXOS (Diet 7), B. circulans ST M53 + 2% AXOS (Diet 8). After four weeks, growth performance and feed conversion ratio significantly improved in fish fed diet 7. Innate immune responses of fish were boosted with both AXOS and probiotic diets, however synergistic effects of AXOS and probiotic diets were only observed for phagocytic and alternative complement activity. Phagocytic and respiratory burst activity of fish macrophage increased in fish fed diet 2 and 7, while humoral immune responses only increased in fish fed diet 7. Pyrosequencing analysis (16S rDNA) of the hindgut microbiota demonstrated that AXOS improved the colonization or/and growth capacity of L. lactis, as a higher relative abundance of L. lactis was observed in fish receiving diet 7. However, no observable colonization of B. circulans was found in the hindgut of fish fed diet 5 or 8, containing this bacterium. The dietary L. lactis ST G45 + 2% AXOS caused significant alterations in the intestinal microbiota by significantly decreasing in bacterial diversity, demonstrated by the fall in richness and Shannon diversity, and improved growth performance and boosted immune responses of Siberian sturgeon

Low prevalence of methicillin-resistant Staphylococcus aureus (MRSA) at hospital admission in the Netherlands: the value of search and destroy and restrictive antibiotic use.

In the Netherlands, less than 1% of clinical isolates of Staphylococcus aureus are methicillin-resistant (MRSA). A national search and destroy policy prevents MRSA from becoming endemic. Some MRSA outbreaks cannot be related to patients at risk for MRSA carriage. This study was designed to measure the prevalence of MRSA among patients without risk factors for MRSA carriage at the time of admission to the hospital. In four Dutch hospitals, patients admitted to non-surgical departments in the period 1999-2000 were screened for MRSA nasal carriage. Nasal swabs were streaked on 5% sheep blood agar (BA), submerged in a selective broth, and incubated for two to three days at 35 degrees C. Colonies suspected of being S. aureus were identified with an agglutination test. Susceptibility testing was performed by an automated system and additional oxacillin disk diffusion. Methicillin resistance was confirmed by a DNA hybridization test and mecA PCR. MRSA strains were genotyped by pulsed-field gel electrophoresis (PFGE). Twenty-four percent (2332/9859) of the patients were S. aureus nasal carriers. Only three (0.03%) patients were MRSA carriers. These patients were not repatriated, nor known to be MRSA carriers before screening. Genotyping revealed that the strains were not clonally related and were not related to MRSA outbreaks in the hospital where the patients were admitted. We conclude that at routine admission to a Dutch hospital (excluding high-risk foreign admissions) the MRSA prevalence is low (0.03%), due to the Dutch search and destroy policy and restrictive antibiotic prescribing