Spondyloepimetaphyseal dysplasia with joint laxity (Beighton type): A unique South African disorder

Spondyloepimetaphyseal dysplasia with joint laxity (SEMD-JL) is an autosomal recessive skeletal dysplasia in which stunted stature, articular hypermobility and spinal malalignment are the major manifestations. Structural cardiac abnormalities are sometimes present. Approximately 30 affected children have been recognised previously in the Afrikaans-speaking community in South Africa, and in several, mutations in the B3GALT6 gene have been incriminated. In this article, case details of three additional affected children in two families are documented, and four additional families are mentioned. The Pierre-Robin sequence and unilateral renal agenesis are previously unreported concomitants. The mutational status where known is recorde

Ultrastructural Assessment of Lesion Development in the Collared Rabbit Carotid Artery Model

Cellular reactions associated with the formation of lesions generated in the carotid artery of rabbits fed either normal or high cholesterol diets by the placement of a flexible, silastic collar around the artery, were studied by electron microscopy. Endothelial cells remained as a monolayer throughout lesion development. The endothelial cell surface in both experimental and sham operated carotids, 4 hours and 8 hours after the initiation of the experiments, were covered with platelets and leukocytes. Neutrophils were present until 7 days in the arteries from within the collar of animals maintained on a normal diet, but only to 1 day in the cholesterol-fed animals. Neutrophils were observed within the medial layer. Few monocytes were identified. An intimal lesion had formed after 7 days in both groups of animals. Macrophage-like cells and foam cells were identified in the cholesterol-fed animals. The size of the lesion increased up to 56 days in animals maintained on a high cholesterol diet, but regression occurred after the 14-days sample in those animals on a normal diet. Concurrently a proportion of the smooth muscle cells changed from contractile to synthetic phenotype within the intimal and medial region of the collared artery of both high cholesterol and normocholesterolaemic animals. Lesions did not form in the contralateral, sham operated arteries

The origins of individual differences in how learning is expressed in rats: a general-process perspective

Laboratory rats can exhibit marked, qualitative individual differences in the form of acquired behaviors. For example, when exposed to a signal-reinforcer relationship some rats show marked and consistent changes in sign-tracking (interacting with the signal; e.g., a lever) and others show marked and consistent changes in goal-tracking (interacting with the location of the predicted reinforcer; e.g., the food well). Here, stable individual differences in rats' sign-tracking and goal-tracking emerged over the course of training, but these differences did not generalize across different signal-reinforcer relationships (Experiment 1). This selectivity suggests that individual differences in sign- and goal-tracking reflect differences in the value placed on individual reinforcers. Two findings provide direct support for this interpretation: the palatability of a reinforcer (as measured by an analysis of lick-cluster size) was positively correlated with goal-tracking (and negatively correlated with sign-tracking); and sating rats with a reinforcer affected goal-tracking but not sign-tracking (Experiment 2). These results indicate that the observed individual differences in sign- and goal-tracking behavior arise from the interaction between the palatability or value of the reinforcer and processes of association as opposed to dispositional differences (e.g., in sensory processes, 'temperament,' or response repertoire)

Attractor Metadynamics in Adapting Neural Networks

Slow adaption processes, like synaptic and intrinsic plasticity, abound in the brain and shape the landscape for the neural dynamics occurring on substantially faster timescales. At any given time the network is characterized by a set of internal parameters, which are adapting continuously, albeit slowly. This set of parameters defines the number and the location of the respective adiabatic attractors. The slow evolution of network parameters hence induces an evolving attractor landscape, a process which we term attractor metadynamics. We study the nature of the metadynamics of the attractor landscape for several continuous-time autonomous model networks. We find both first- and second-order changes in the location of adiabatic attractors and argue that the study of the continuously evolving attractor landscape constitutes a powerful tool for understanding the overall development of the neural dynamics

Impact of the COVID-19 pandemic on cancer assessment in primary care: a qualitative study of GP views

Funding This research was co-funded by the National Institute for Health Research (NIHR) Policy Research Programme, conducted through the Policy Research Unit in Cancer Awareness, Screening and Early Diagnosis, PR-PRU-1217–21601. The views expressed are those of the authors and not necessarily those of the NIHR or the Department of Health and Social Care. The study was also supported by the CanTest Collaborative funded by Cancer Research UK C8640/A23385, of which Fiona Walter is director, Richard Neal is associate director, Suzanne Scott is co-investigator, and Natalia Calanzani and Stephanie Honey are researchers. The funders of the study had no role in study design, data collection, data analysis, data interpretation, or writing of the report.Peer reviewedPublisher PD

A novel algorithm for dynamic student profile adaptation based on learning styles

The file attached to this record is the author's final peer reviewed version. The Publisher's final version can be found by following the DOI link.E-learning recommendation systems are used to enhance student performance and knowledge by providing tailor- made services based on the students’ preferences and learning styles, which are typically stored in student profiles. For such systems to remain effective, the profiles need to be able to adapt and reflect the students’ changing behaviour. In this paper, we introduce new algorithms that are designed to track student learning behaviour patterns, capture their learning styles, and maintain dynamic student profiles within a recommendation system (RS). This paper also proposes a new method to extract features that characterise student behaviour to identify students’ learning styles with respect to the Felder-Silverman learning style model (FSLSM). In order to test the efficiency of the proposed algorithm, we present a series of experiments that use a dataset of real students to demonstrate how our proposed algorithm can effectively model a dynamic student profile and adapt to different student learning behaviour. The results revealed that the students could effectively increase their learning efficiency and quality for the courses when the learning styles are identified, and proper recommendations are made by using our method

Modulation of Myocardial Mitochondrial Mechanisms during severe Polymicrobial Sepsis in the Rat

Background: We tested the hypothesis that 5-Hydroxydecanoic acid (5HD), a putative mitoKATP channel blocker, will reverse sepsis-induced cardiodynamic and adult rat ventricular myocyte (ARVM) contractile dysfunction, restore mitochondrial membrane permeability alterations and improve survival. Methodology/Principal Findings: Male Sprague-Dawley rats (350-400 g) were made septic using 400 mg/kg cecal inoculum, ip. Sham animals received 5% dextrose water, ip. The Voltage Dependent Anion Channels (VDAC1), Bax and cytochrome C levels were determined in isolated single ARVMs obtained from sham and septic rat heart. Mitochondria and cytosolic fractions were isolated from ARVMs treated with norepinephrine (NE, 10 µmoles) in the presence/absence of 5HD (100 µmoles). A continuous infusion of 5HD using an Alzet pump reversed sepsis-induced mortality when administered at the time of induction of sepsis (-40%) and at 6 hr post-sepsis (-20%). Electrocardiography revealed that 5HD reversed sepsis-induced decrease in the average ejection fraction, Simpsons+m Mode (53.5±2.5 in sepsis and 69.2±1.2 at 24 hr in sepsis+5HD vs. 79.9±1.5 basal group) and cardiac output (63.3±1.2 mL/min sepsis and 79.3±3.9 mL/min at 24 hr in sepsis+5HD vs. 85.8±1.5 mL/min basal group). The treatment of ARVMs with 5HD also reversed sepsis-induced depressed contractility in both the vehicle and NE-treated groups. Sepsis produced a significant downregulation of VDAC1, and upregulation of Bax levels, along with mitochondrial membrane potential collapse in ARVMs. Pretreatment of septic ARVMs with 5HD blocked a NE-induced decrease in the VDAC1 and release of cytochrome C. Conclusion: The data suggest that Bax activation is an upstream event that may precede the opening of the mitoKATP channels in sepsis. We concluded that mitoKATP channel inhibition via decreased mitochondrial membrane potential and reduced release of cytochrome C provided protection against sepsis-induced ARVM and myocardial contractile dysfunction. © 2011 Chopra et al

Maximising Social Interactions and Effectiveness within Distance Learning Courses: Cases from Construction

Advanced Internet technologies have revolutionised the delivery of distance learning education. As a result, the physical proximity between learners and the learning providers has become less important. However, whilst the pervasiveness of these technological developments has reached unprecedented levels, critics argue that the student learning experience is still not as effective as conventional face-to-face delivery. In this regard, surveys of distance learning courses reveal that there is often a lack of social interaction attributed to this method of delivery, which tends to leave learners feeling isolated due to a lack of engagement, direction, guidance and support by the tutor. This paper defines and conceptualises this phenomenon by investigating the extent to which distance-learning programmes provide the social interactions of an equivalent traditional classroom setting. In this respect, two distance learning case studies were investigated, covering the UK and Slovenian markets respectively. Research findings identified that delivery success is strongly dependent on the particular context to which the specific distance learning course is designed, structured and augmented. It is therefore recommended that designers of distance learning courses should balance the tensions and nuances associated with commercial viability and pedagogic effectiveness