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

Taking ownership of technology: Lecturers as LMS learners

Our paper presents the findings from a study of personalised support in the use of the Learning Management System (LMS) to lecturers at the Faculty of Medical and Health Sciences, University of Auckland. Our study indicates that personalised support is an effective means of supporting some lecturers as they learn about the use of technology for teaching. We conclude our paper by considering the value of this research for the Faculty.published_or_final_versionThe 23rd Annual Conference of the Australasian Society for Computers in Learning in Tertiary Education: Who’s Learning? Whose Technology?, Sydney, Australia, 3-6 December 2006. In Proceedings of the 23rd Annual Ascilite Conference: Who’s learning? Whose technology?, 2006, v. 1, p. 213-21

Chronic Brucella Pyelonephritis with Calcification: Short Review of the Literature and Report of a Case

A CAJM article on Chronic Brucella Pyelonephritis.The localisation of brucellosis in the urinary tract is an infrequently reported condition. The paucity of recorded cases may be due to the rarity of the condition or to the fact that the profession as a whole is not sufficiently aware of the possibility of urinary tract involvement in brucellosis. Having recently investigated a patient who is suffering from pyelonephritis and cystitis occurring as a complication of brucellosis, we were prompted to publish his case record with the aim of calling the attention of the profession to the various aspects of this disease and emphasising its localisation in the genitourinary system

A Peer-To-Peer Data Processing Infrastructure That Operates On The Largest Scale

In the corporate network, information may be exchanged throughout different businesses, making it simpler for those with common interests to collaborate. With its help, firms may be able to reduce overhead costs and increase revenue. As data is exchanged and processed across firms, it increases the complexity of implementing a scalable, high-performing, and secure data management system. This article presents BP++, an expansion of the BestPeer P2P data management platform that offers elastic data sharing services for cloud-based business network applications. BP++ integrates cloud computing, databases, and P2P technologies to provide its members with data sharing services under the well-known pay-as-you-go pricing model. For our BP++ tests, we make use of Amazon's EC2 cloud infrastructure. Benchmarks show that BP++ outperforms the recently proposed HadoopDB large-scale data processing solution when both systems are employed to handle typical business network demands. The results demonstrate that BP++ is quite efficient, with throughput scaling practically linearly with the number of peer nodes

Elaboration of a model of Pavlovian learning and performance: HeiDI

The model elaborated here adapts the influential pooled error term, first described by Allan R. Wagner and his colleague Robert A. Rescorla, to govern the formation of reciprocal associations between any pair of stimuli that are presented on a given trial. In the context of Pavlovian conditioning, these stimuli include various conditioned and unconditioned stimuli. This elaboration enables the model to deal with cue competition phenomena, including the relative validity effect, and evidence implicating separate error terms and attentional processes in association formation. The model also includes a performance rule, which provides a natural basis for (individual) variation in the strength and nature of conditioned behaviors that are observed in Pavlovian conditioning procedures. The new model thereby begins to address theoretical and empirical issues that were apparent when the Rescorla-Wagner model was first described, together with research inspired by the model over ensuing 50 years

Individual differences in the nature of conditioned behavior across a conditioned stimulus: adaptation and application of a model

Pavlovian conditioning procedures produce marked individual differences in the form of conditioned behavior. For example, when rats are given conditioning trials in which the temporary insertion of a lever into an operant chamber (the conditioned stimulus, CS) is paired with the delivery of food (the unconditioned stimulus, US), they exhibit knowledge of the leverfood relationship in different ways. For some rats (known as sign-trackers) interactions with the lever dominate, while for others (goal-trackers) approaching the food well dominates. A formal model of Pavlovian conditioning (HeiDI) attributes such individual differences in behavior to variations in the perceived salience of the CS and US. An application of the model in which the perceived salience of the CS declines (i.e., adapts) across its duration, predicts changes in these individual differences within the presentation of the CS: The sign-tracking bias is predicted to decline and goal-tracking bias is predicted to increase across the presentation of a lever. The accuracy of these predictions was confirmed though analysis of archival data from female and male rats

Distributions of particulate Heme <i>b</i> in the Atlantic and Southern Oceans— Implications for electron transport in phytoplankton

Concentrations of heme b, the iron-containing component of b-type hemoproteins, ranged from?<?0.4 to 5.3 pM with an average of 1.18?±?0.8 pM (± 1s; n?=?86) in the Iceland Basin (IB), from?<?0.4 to 19.1 pM with an average of 2.24?±?1.67 pM (n?=?269) in the tropical northeast Atlantic (TNA) and from 0.6 to 21 pM with an average of 5.1?±?4.8 pM (n?=?34) in the Scotia Sea (SS). Heme b concentrations were enhanced in the photic zone and decreased with depth. Heme b concentrations correlated positively with chlorophyll a (chl a) in the TNA (r?=?0.41, p?<?0.01, n?=?269). Heme b did not correlate with chl a in the IB or SS. In the IB and SS, stations with high-chlorophyll and low-nutrient (Fe and/or Si) concentrations exhibited low heme b concentrations relative to particulate organic carbon (< 0.1?µmol?mol-1), and high chl a:heme b ratios (> 500). High chl a:heme b ratios resulted from relative decreases in heme b, suggesting proteins such as cytochrome b6f, the core complex of photosystem II, and eukaryotic nitrate reductase were depleted relative to proteins containing chlorophyll such as the eukaryotic light-harvesting antenna. Relative variations in heme b, particulate organic carbon, and chl a can thus be indicative of a physiological response of the phytoplankton community to the prevailing growth conditions, within the context of large-scale changes in phytoplankton community composition