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Darier disease : more than skin deep
No full textDarier disease (DD) is a severe, inherited dermatological disorder with a characteristic clinical and histopathological appearance. It is caused by mutations in the ATP2A2 gene, encoding a Ca2+ pump in the endoplasmic reticulum, Sarco/Endoplasmic Reticulum Ca2+-ATPase 2 (SERCA2), which has been found to be an essential regulator of cellular Ca2+ homeostasis. SERCA2 is expressed in all cells of the body, not just the skin, and has been implicated in physiological and pathophysiological mechanisms in several organs, thus rendering the occurrence of extra-dermal presentations plausible. DD has so far only been associated with brain-related disorders. The overall aim of this thesis was to investigate comorbidities associated with DD.In study I, II and III, experimental, matched case-control designs were used to evaluate broad cognitive function, heart disease and diabetes in individuals with DD compared with matched healthy controls. Individuals with DD were found to exhibit a widespread, significant impairment in cognitive function assessed with the Cambridge Neuropsychological Test Automated Battery, evaluating general cognitive function. A possible defect in serum lipid handling was also seen, as well as evidence of pancreatic β-cell dysfunction indicating a metabolic state seen during the development of type 2 diabetes.In study II and IV, a population-based cohort design, comparing data on individuals with DD taken from the National Patient Register with matched comparison subjects from the Total Population Register, was used. Individuals with DD displayed a 59% risk increase of heart failure (risk ratio [RR] 1.59, confidence interval [CI] 1.16-2.19) and a 32% risk increase of any heart diagnosis (RR 1.32, CI 1.02-1.70). Female DD patients had a 59% risk increase of any heart diagnosis (RR 1.59, CI 1.13-2.25). The first heart diagnosis also occurred almost seven years earlier in individuals with DD (70.0 vs. 76.9 years). A 74% increased risk of type 1 diabetes diagnosis was also seen (RR 1.74, CI 1.13-2.69).In study V, immune cell markers were evaluated in a small cohort of DD patients. The values of CD19+ B cells were found to be lower than the normal laboratory range on average, which together with the evidence of the importance of SERCA2 in B cell development indicates a possible immune system defect. This finding can help explain the recurring secondary infections often troubling individuals with DD.In conclusion, DD has been found to be associated with impaired cognitive function, heart disease and diabetes, as well as a possible defect in the immune system. The range of comorbidities associated with DD has been expanded to include several organs, resulting in the confident conclusion that DD should be viewed as a systemic disorder. This has several important implications, both for the individual patient and in routine care for this group of patients. Looking forward, it will quite possibly influence the way DD is treated.List of scientific papersI. Philip Curman, Johanna Bern, Linnea Sand, Martin Cederlöf, Etty Bachar-Wikström, Jakob D. Wikström. Patients with Darier Disease Exhibit Cognitive Impairment while Patients with Hailey-Hailey Disease Do Not: An Experimental, Matched Case-control Study. Acta Derm Venereol. 2021. 101(6). https://doi.org/10.2340/00015555-3818 II. Etty Bachar-Wikstrom, Philip Curman, Tara Ahanian, Ivone U.S. Leong, Henrik Larsson, Martin Cederlöf, Jakob D. Wikstrom. Darier disease is associated with heart failure: a cross-sectional case-control and population based study. Sci Rep. 2020. 10(1). https://doi.org/10.1038/s41598-020-63832-9 III. Tara Ahanian*, Philip Curman*, Ivone U.S. Leong, Kerstin Brismar, Etty Bachar- Wikstrom, Martin Cederlöf, Jakob D. Wikstrom. Metabolic phenotype in Darier disease: a cross-sectional clinical study. Diabetol Metab Syndr. 2020. 12. * These authors contributed equally to this work. https://doi.org/10.1186/s13098-020-0520-0 IV. Martin Cederlöf, Philip Curman, Tara Ahanian, Ivone U.S. Leong, Kerstin Brismar, Etty Bachar-Wikstrom, Jakob D. Wikstrom. Darier disease is associated with type 1 diabetes: Findings from a population-based cohort study. J Am Acad Dermatol. 2019. 81(6). https://doi.org/10.1016/j.jaad.2019.05.087 V. Chun-Chin Chen*, Bo-Ruei Chen*, Yinan Wang, Philip Curman, Helen A. Beilinson, Ryan M. Brecht, Catherine C. Liu, Ryan J. Farrell, Jaime de Juan-Sanz, Louis-Marie Charbonnier, Shingo Kajimura, Timothy A. Ryan, David G. Schatz, Talal A. Chatila, Jakob D. Wikstrom, Jessica K. Tyler, Barry P. Sleckman. Sarco/endoplasmic reticulum Ca2+-ATPase (SERCA) activity is required for V(D)J recombination. J Exp Med. 2021. 218(8). * These authors contributed equally to this work. https://doi.org/10.1084/jem.20201708 </p
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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