1,720,964 research outputs found
Exercise physiology applied to the evaluation of exercise tolerance in patients with late-onset pompe disease
Get PDFPompe disease is a rare inherited metabolic disorder due to the deficiency of the lysosomal enzyme alfa glucosidase, with consequent abnormal glycogen accumulation in muscle fibres. The late-onset form (LOPD) is characterised by a progressive motor and respiratory impairment. The specific enzyme replacement therapy (ERT), has been proved to stabilise muscle functions, but its effect after many years of treatment seems to be limited, especially for those patients who are already compromised at baseline. Exercise intolerance is one of the first clinical hallmarks affecting LOPD patients' quality of life. Functional evaluation of exercise tolerance is a recently tested method to assess clinical LOPD outcome. Before the introduction of ERT, the positive effects of diet and exercise training had already been demonstrated in LOPD, but the effect of these interventions on patients treated with ERT is still debated and poorly known.
This thesis is articulated into 3 studies, the first investigate the long term (more than 5 years) effects of ERT on a cohort of patients with the juvenile form of LOPD. The second investigates the acute effects of ERT on the main variables linked to exercise tolerance, dividing patients by clinical severity and level of habitual physical activity, and comparing results obtained from exercise tolerance test with those from standard six minutes walking test (6MWT). The third study is still ongoing, and investigates if a specific program of exercise training, alone or combined to hyperproteic diet, can ameliorate exercise tolerance in LOPD patients chronically treated with ERT.
For patients’ clinical evaluation the following parameters were mainly used: serum muscle enzymes (AST, ALT, CPK, LDH ), muscle strength (by dynamometer), motor function (6MWT, Walton Scale), neurologic functional tests, pulmonary function tests (standard spirometry, Maximal Inspiratory Pressure, Maximal Expiratory Pressure).
The functional evaluation of exercise tolerance was conducted on a cycle-ergometer and a specific exercise protocol adapted to patients was developed. The main variable of exercise tolerance assessed were: maximal aerobic power; gas exchange threshold; kinetics of pulmonary gas exchange during constant work-rate exercises; skeletal muscle fractional O2 extraction by near-infrared spectroscopy; heart rate, pulmonary ventilation and gas exchange ratio during constant work-rate exercise; rates of perceived exertion; time to exhaustion.
The results of these studies demonstrate: 1. Patients with the juvenile form of Pompe disease show a stabilization of motor and respiratory functions after 5 years of regular treatment with ERT. Patients who started therapy in an asymptomatic phase were still free of symptoms after 6 years of follow up, suggesting that ERT is more efficient if started at early stages of the disease. 2. The exercise tolerance test is not acutely influenced by ERT, its main utility in clinical practice is in the long term follow up of patients, in which it seems to be more sensible than the 6MWT. The habitual physical activity seems to improve the exercise tolerance, regardless the disease severity. 3. Aerobic exercise training may ameliorate skeletal muscle force, maximal aerobic capacity and peripheral oxygen extraction in LOPD patients on chronic ERT treatmen
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Get PDF“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Get PDFWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Dispelling the Myths Behind First-author Citation Counts
Get PDFWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
koamabayili/VECTRON-author-checklist: VECTRON author checklist
No full textWe have done our best to complete the author checklist relating to the use of animals in the hut study. Note that the objective for the hut study was to evaluate the IRS treatment applications for residual efficacy against Anopheles mosquitoes, including the local An. coluzzii mosquito population. Cows were only used to attract mosquitoes into the huts and no tests were carried out directly on the cows. The author checklist is intended for use with studies where experiments are carried out on animals, which is why we have had such difficulty in completing this for the hut study, as many of the questions do not relate to how the cows were used
Author-wise bibliometric analysis based on entropy.
No full textAuthor-wise bibliometric analysis based on entropy.</p
Author Under Sail The Imagination of Jack London, 1893-1902
No full textIn Author Under Sail, Jay Williams offers the first complete literary biography of Jack London as a professional writer engaged in the labor of writing. It examines the authorial imagination in London's work, the use of imagination in both his fiction and nonfiction, and the ways he defined imagination in the creative process in his business dealings with his publishers, editors, and agents. In this first volume of a two-volume biography, Williams traverses the years 1893 to 1902, from London's "Story of a Typhoon" to The People of the Abyss. The Jack London who emerges in the pages of Author Under Sail is a writer whose partnership with publishers, most notably his productive alliance with George Brett of Macmillan, was one of the most formative in American literary history. London pioneered many author models during the heyday of realism and naturalism, blurring the boundaries of these popular genres by focusing on absorption and theatricality and the representation of the seen and unseen. London created an impassioned, sincere, and extremely personal realism unlike that of other American writers of the time. Author Under Sail is a literary tour de force that reveals the full range of London as writer, creative citizen, and entrepreneur at the same time it sheds light on the maverick side of machine-age literature.Intro -- Title Page -- Copyright Page -- Dedication -- Contents -- Acknowledgments -- Introduction -- 1. Spirit Truth -- 2. From Absorption to Theatricality and Back Again -- 3. "I Will Build a New Present" -- 4. Sons as Authors -- 5. Fathers as Publishers -- 6. The Daughter as Author -- 7. Lovers as Authors -- 8. At Sea with the Family -- 9. Yellow News, Yellow Stories -- 10. The Return Home -- Notes -- Bibliography -- Index -- About Jay WilliamsIn Author Under Sail, Jay Williams offers the first complete literary biography of Jack London as a professional writer engaged in the labor of writing. It examines the authorial imagination in London's work, the use of imagination in both his fiction and nonfiction, and the ways he defined imagination in the creative process in his business dealings with his publishers, editors, and agents. In this first volume of a two-volume biography, Williams traverses the years 1893 to 1902, from London's "Story of a Typhoon" to The People of the Abyss. The Jack London who emerges in the pages of Author Under Sail is a writer whose partnership with publishers, most notably his productive alliance with George Brett of Macmillan, was one of the most formative in American literary history. London pioneered many author models during the heyday of realism and naturalism, blurring the boundaries of these popular genres by focusing on absorption and theatricality and the representation of the seen and unseen. London created an impassioned, sincere, and extremely personal realism unlike that of other American writers of the time. Author Under Sail is a literary tour de force that reveals the full range of London as writer, creative citizen, and entrepreneur at the same time it sheds light on the maverick side of machine-age literature.Description based on publisher supplied metadata and other sources.Electronic reproduction. Ann Arbor, Michigan : ProQuest Ebook Central, YYYY. Available via World Wide Web. Access may be limited to ProQuest Ebook Central affiliated libraries
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