Gross motor abilities and interventions in girls and women with Rett syndrome: A literature review ; Longitudinal video analysis of gross motor abilities of girls and women with Rett syndrome

Objective: To explore research relevant to an understanding of gross motor abilities and highlight possible directions for gross motor intervention in girls and women with Rett syndrome. A secondary objective was to describe mouse model research which has the potential to add to an understanding of gross motor abilities in this population. Methods: Electronic searches of five databases, manual searches of an external resource library and manual searches of reference lists were undertaken. The key words imputed during these searches included; mobility, Rett syndrome, functioning, mouse model, therapy and intervention. Search terms were truncated, exploded and adjusted to achieve optimum results. A narrative review was possible. Results: The searches of the literature yielded research which will be discussed under the headings; phenotype-genotype correlations, gross motor functioning, therapy interventions, involving both gross motor interventions in girls and women with Rett syndrome and mouse model research. The research found that most girls/women with Rett syndrome can sit independently, approximately half can walk and many have difficulties with transitional movements. More complex gross motor skills, such as transitions, walking on a slope and stepping over an obstacle, have been highlighted as especially difficult for this population. Video analysis is an emerging methodology in this area and has the potential to ·provide better observational data, to measure change, investigate gross motor abilities and evaluate the effectiveness of gross motor interventions. Mouse model research has investigated environmental enrichment as a treatment paradigm resulting in amelioration of gross motor deterioration. Similarly, increasing the expression of BDNF in mice with MeCP2 mutants has resulted in reduced locomotor deficit. Conclusion: Further longitudinal and cross-sectional studies with rigorous design and larger sample sizes are required in order to guide therapeutic gross motor intervention in girls and women with Rett syndrome. Purpose: Rett syndrome is a rare neurological disorder often associated with a mutation in the MECP 2 gene. It results in severe physical and intellectual disability with a gradual decrease in acquired gross motor abilities. This study explored changes in gross motor abilities over three years in girls/women with Rett syndrome, recruited from a population-based data base. The relationships between these changes and age and genotype were investigated. Method: Families participating in the Australian Rett Syndrome Database were invited to participate in a video study. Ninety-nine families provided a video in 2004 and 70 of these cases submitted a second video in 2007. Gross motor data for the two time points were scored through the use of an assessment tool based on the Gross Motor Function Measure. Results: The level of general gross motor skills decreased in 58 (82.9%) and increased in 12 (17.1 %) cases (mean decrease in z-score 0.50 ± 0.59). The level of complex gross motor skills decreased in 67 (95.7%) and increased in 3 (4.2%) cases (mean decrease in z-score 1.58 ± 1.11). General motor skills declined for cases in each of the four age-groups. Compared to the girls who were younger than 8 years, the decrease in complex motor skills was greater for girls aged 13:S19 years (P=0.021) and women \u3e19 years (P=0.071). Conclusion: Over a 3-year period, there was a small amount of deterioration in general gross motor skills for girls of all ages and a larger deterioration in complex gross motor skills during the teenage years. This detailed understanding of the characteristics of declining gross motor skills in Rett syndrome could contribute to the development of strategies to ameliorate these trends

Public archaeology with a Doukhobor descendant community

Fleeing religious persecution in Russia, large groups of Doukhobors arrived in Canada in 1899, and settled in Saskatchewan. Today archaeology can serve a pivotal role in the Doukhobor community's efforts to reclaim and celebrate its past. A partnership between the Doukhobor community and the Saskatchewan archaeological community, created "The Doukhobor Pit-House Public Archaeology Project." The project featured the participation of Doukhobor descendants in the excavation of two Doukhobor sites. This provided an opportunity not only to provide a beneficial experience for the community but also to study how a public archaeology project is formed and operates to a successful finish. Using qualitative data including questionnaires, daily journals and interviews, this project evaluated the impact the archaeological experience had upon the changing Doukhobor community, by discerning the project's successes, failures and benefits. The results of the evaluation provides practical information for future public archaeology projects and wider implications for the discipline's relationship with the public. The analysis also provides a deeper understanding of how archaeology can provide Canadian communities with a voice in the creation and maintenance of their past for the betterment of future generations and the wider provincial and national community

Library Publishing Curriculum Textbook

In the original, modular curriculum (2018) on which this textbook is based, each unit of the Library Publishing Curriculum contained an instructor’s guide, narrative, a slideshow with talking notes, bibliographies, supplemental material, and activities for use in a physical or virtual classroom for workshops and courses. This textbook version, produced in 2021, adapts the original narrative as the primary content (with very little additional editing) and incorporates the bibliographies, appendices, and images from the slideshow into a linear reading and learning experience for use by librarians or students learning on their own or as part of a classroom learning experience. The LPC hopes others use and extend this CC-BY version into even more learning opportunities to help create a more equitable publishing ecosystem

Cost-effectiveness analysis of radiofrequency ablation versus drugs for the treatment of a trial fibrillation in the South African population

Thesis (PhD)--Stellenbosch University, 2021.ENGLISH SUMMARY : Atrial fibrillation is the most commonly found and sustained arrhythmia. It affects about 1% of the total population and is found in more than one in ten in the elderly. The prevalence is increasing with the aging population. Patients with atrial fibrillation are at an increased risk of heart failure and all-cause mortality, and have a fivefold increased risk of stroke. Atrial fibrillation is associated with debilitating symptoms and an impaired quality of life. The restoration and maintenance of sinus rhythm is favourable and the current guidelines recommend the use of both rate and rhythm control strategies, which involve the prescription of anti-arrhythmic drug therapy. These anti-arrhythmic drugs are frequently ineffective, with large studies showing that atrial fibrillation returns in as many as 85% of patients within one year. These drugs are also associated with adverse events, occasionally serious, which may lead to hospitalisation. Catheter ablation (a minimally invasive procedure), has over the past decade revolutionised the treatment of atrial fibrillation. The 2010 European Society of Cardiology guidelines recommend catheter ablation for paroxysmal atrial fibrillation as a class IIa recommendation with level of evidence “A”. Current literature indicates that patients with atrial fibrillation who undergo pulmonary vein ablation have a significantly lower risk of death, stroke and dementia compared to patients with atrial fibrillation who are not treated with ablation, while stroke and dementia is similar to that of the general population. This study used a decision tree analysis, a Markov model and Monte Carlo simulation to calculate the cost-effectiveness of catheter ablation versus commonly used anti-arrhythmic drugs for the treatment of paroxysmal atrial fibrillation. Input into the model was founded on an extensive literature review, interviews with local electrophysiologists and a sample of real patient data, which examined the costs associated with among others, the length of hospital stay and the cost of the procedure. The model simulated 1 000 patients receiving either pulmonary vein isolation through radiofrequency ablation or anti-arrhythmic drugs and the following variables were measured, QALYs, average cost, incremental costs, average effectiveness, incremental effectiveness, average length of stay in hospital for complications, relative risk of death for radiofrequency catheter ablation versus anti-arrhythmic drugs and also the net monetary benefits. A total of fourteen variables were tested and sensitivity analyses were performed on each. It was found that in all but two cases, pulmonary vein isolation with radiofrequency catheter ablation dominated over anti-arrhythmic drug therapy as being more cost-effective for the management of paroxysmal atrial fibrillation. Finally, it was determined that pulmonary vein isolation with radiofrequency catheter ablation should be considered as a first line therapy for patients with paroxysmal atrial fibrillation in South Africa.AFRIKAANSE OPSOMMING : Atriale fibrillasie is die mees algemene en volgehoue aritmie. Dit raak ongeveer 1% van die bevolking. Onder bejaardes is die voorkoms egter soveel as 10%, wat beteken dat die toestand toeneem in verhouding met die verouderende bevolking. Atriale fibrillasie verhoog ’n pasiënt se risiko vir beroerte vyfvoudig, tesame met hartversaking en alle-oorsake mortaliteit. Die toestand se uitmergelende simptome verlaag ook lewenskwaliteit. Die kanse vir die herstel en instandhouding van sinusritme is positief. Bestaande riglyne beveel dus die gebruik van ritme- of spoed-beheer medikasie aan, wat beteken dat anti-aritmie medikasie voorgeskryf word. Dit is egter dikwels oneffektief en verskeie omvattende studies het bewys dat tot 85% van die pasiënte binne een jaar weer ‘n ritmestoornis ervaar. Hierdie middels word ook dikwels verbind met newe-effekte wat ernstig mag wees en tot hospitalisasie mag lei. Kateterablasie (’n minimaal-ingrypende prosedure) het oor die afgelope dekade die behandeling van atriale fibrillasie onherkenbaar verander. Vir pasiënte wat hoogs simptomaties, of paroksismaal, is ten spyte van optimale terapie, beveel die Europese Vereniging van Kardiologie se 2010 riglyne ablasie aan as ‘n klas IIa aanbeveling. Huidige literatuur dui op ‘n laer koers van sterftes, beroerte en demensie onder atriale fibrillasie pasiënte wat atriale fibrillasie ablasie ondergaan, in vergelyking met pasiënte wat met medikasie behandel word. Eersgenoemde het inteendeel dieselfde risikoprofiel as die algemene bevolking. Hierdie studie gebruik ’n keuse-boom analisemodel en ’n waarskynlikheid-Markov model met Monte Carlo-simulasie om die koste-effektiwiteit van kateterablasie met anti-aritmiese medikasie te vergelyk in die behandeling van pasiënte met paroksismale atriale fibrillasie. Die data wat in die model gebruik word, is gebaseer op ’n uitgebreide literatuurstudie, onderhoude met elektrofisioloë, en ’n steekproef van pasiëntdata, en ondersoek, onder andere, die duur van hopitaalverblyf en die koste verbonde aan die prosedure. Die studie maak gebruik van ’n simulasiemodel waar 1 000 pasiënte óf ablasie óf medikasie ontvang het. Die volgende veranderlikes is gemeet: QALYs, gemiddelde koste, toenemende koste, gemiddelde doeltreffendheid, toenemende doeltreffendheid, gemiddelde hospitaalverblyf tydens komplikasies, die relatiewe riskio van sterfte en monetêre voordele. Veertien veranderlikes is getoets en aan sensitiwiteitsanalises onderwerp. In dertien uit die viertien analises was kateterablasie meer koste-effektief as anti-aritmiese medikasie in die behandeling en bestuur van anti-aritmiese medikasie. Die studie kom dus tot die gevolgtrekking dat kateterablasie oorweeg moet word as voorkeurterapie vir pasiënte met paroksismale atriale fibrillasie in Suid-Afrika.Doctora

Production networks in the cultural and creative sector: case studies from the publishing industry (CICERONE report D2.8)

The CICERONE project investigates cultural and creative industries through case study research, with a focus on production networks. This report, part of WP2, examines the publishing industry within this framework. It aims to understand the industry's hidden aspects, address statistical issues in measurement, and explore the industry's transformation and integration of cultural and economic values. The report provides an overview of the production network, explores statistical challenges, and presents qualitative analyses of two case studies. It concludes by highlighting the potential of the Global Production Network (GPN) approach for analyzing, researching, policymaking, and intervening in the European publishing network. The CICERONE project's case study research delves into the publishing industry, investigating its production networks and examining key aspects often unseen by the public. The report addresses statistical challenges in measuring the industry and sheds light on its ongoing transformations and integration of cultural and economic values. It presents an overview of the production network, explores statistical issues, and provides qualitative analyses of two case studies. The report emphasizes the potential of the GPN approach for analyzing and intervening in the European publishing network, ultimately contributing to research, policymaking, and understanding within the industry

A framework for the analysis and evaluation of enterprise models

Bibliography: leaves 264-288.The purpose of this study is the development and validation of a comprehensive framework for the analysis and evaluation of enterprise models. The study starts with an extensive literature review of modelling concepts and an overview of the various reference disciplines concerned with enterprise modelling. This overview is more extensive than usual in order to accommodate readers from different backgrounds. The proposed framework is based on the distinction between the syntactic, semantic and pragmatic model aspects and populated with evaluation criteria drawn from an extensive literature survey. In order to operationalize and empirically validate the framework, an exhaustive survey of enterprise models was conducted. From this survey, an XML database of more than twenty relatively large, publicly available enterprise models was constructed. A strong emphasis was placed on the interdisciplinary nature of this database and models were drawn from ontology research, linguistics, analysis patterns as well as the traditional fields of data modelling, data warehousing and enterprise systems. The resultant database forms the test bed for the detailed framework-based analysis and its public availability should constitute a useful contribution to the modelling research community. The bulk of the research is dedicated to implementing and validating specific analysis techniques to quantify the various model evaluation criteria of the framework. The aim for each of the analysis techniques is that it can, where possible, be automated and generalised to other modelling domains. The syntactic measures and analysis techniques originate largely from the disciplines of systems engineering, graph theory and computer science. Various metrics to measure model hierarchy, architecture and complexity are tested and discussed. It is found that many are not particularly useful or valid for enterprise models. Hence some new measures are proposed to assist with model visualization and an original "model signature" consisting of three key metrics is proposed.Perhaps the most significant contribution ofthe research lies in the development and validation of a significant number of semantic analysis techniques, drawing heavily on current developments in lexicography, linguistics and ontology research. Some novel and interesting techniques are proposed to measure, inter alia, domain coverage, model genericity, quality of documentation, perspicuity and model similarity. Especially model similarity is explored in depth by means of various similarity and clustering algorithms as well as ways to visualize the similarity between models. Finally, a number of pragmatic analyses techniques are applied to the models. These include face validity, degree of use, authority of model author, availability, cost, flexibility, adaptability, model currency, maturity and degree of support. This analysis relies mostly on the searching for and ranking of certain specific information details, often involving a degree of subjective interpretation, although more specific quantitative procedures are suggested for some of the criteria. To aid future researchers, a separate chapter lists some promising analysis techniques that were investigated but found to be problematic from methodological perspective. More interestingly, this chapter also presents a very strong conceptual case on how the proposed framework and the analysis techniques associated vrith its various criteria can be applied to many other information systems research areas. The case is presented on the grounds of the underlying isomorphism between the various research areas and illustrated by suggesting the application of the framework to evaluate web sites, algorithms, software applications, programming languages, system development methodologies and user interfaces