1,721,080 research outputs found
L'œdème angioneurotique héréditaire (à propos d'un cas et revue de la littérature)
No full textPARIS7-Xavier Bichat (751182101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF
SYNDROME DE RAYNAUD CHEZ L'ENFANT; APPORT DE LA CAPILLAROSCOPIE.
No full textAIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF
Intérêt d'un traitement par Paminodrate intraveineux dans la prise en charge de l' OCMR réfractaire de l' enfant
No full textL ostéomyélite chronique multifocale récurrente (OCMR) est une maladie pédiatrique inflammatoire rare. Le traitement de première ligne utilise les anti-inflammatoires non stéroïdiens (AINS). Le traitement des OCMR réfractaires n est pas codifié. Le pamidronate, un biphosphonate, semble être efficace sur la douleur et les lésions objectivées en IRM. Des effets secondaires potentiels à long terme peuvent rendre son usage pédiatrique délicat. Ce travail étudie les indications, l efficacité et la tolérance du pamidronate chez 13 Nous avons analysé rétrospectivement les dossiers de 13 patients suivis pour OCMR, en décrivant les motifs d utilisation du pamidronate, ses modalités d administration, son efficacité clinique et radiologique, et sa tolérance. Treize patients (4M, 9F) ont reçu du pamidronate de novembre 2004 à janvier 2013. L âge médian au diagnostic était de 10 ans (6-14 ans). La voie d administration était intraveineuse, par cure de 3 jours. Onze patients ont reçu une seule cure, 1 patient 4 cures et 1 patient 6 cures. Le motif principal était la douleur résistante aux AINS et aux antalgiques. 11 patients rapportaient une EVA à 0/10 en moyenne 36 heures post-administration. Les douleurs ont persisté pour 1 patient et disparu après 3 semaines pour un autre. La tuméfaction claviculaire a disparu chez 1/5 patients et nettement diminué chez 4/5 patients. La durée médiane entre deux crises douloureuses était de 10 mois (1-21.6). Huit patients ont eu une IRM de contrôle montrant une diminution des lésions (n=4) après un délai médian de 29.7 mois après le début du pamidronate. Les effets secondaires étaient : fièvre précoce (n=8) dont 5 syndromes grippaux, hypocalcémie (n=12, sévère n=5 dont 2 symptomatiques). Le pamidronate apparait être un traitement efficace et bien toléré de l OCMR. Des essais cliniques contrôlés randomisés semblent nécessaires pour confirmer ces constatations.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF
Validation of the PEDiatric Behçet’s Disease classification criteria: an evidence-based approach
No full textObjectives: To validate the PEDiatric Behcet's Disease classification criteria (PEDBD) with an evidence-based approach.Methods: A total of 210 pediatric patients [70 Behcet's disease (BD), 40 periodic fever, aphthous stomatitis, pharyngitis, adenitis, 35 familial Mediterranean fever, 26 hyper-IgD syndrome, 22 TNF-receptor associated periodic fever syndrome, 17 undefined recurrent fevers] were randomly selected from the Eurofever Registry. A set of 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients. Using the table consensus as gold standard (agreement >= 80%), the PEDBD, ISG and ICBD criteria were applied to BD patients and to confounding diseases with other autoinflammatory conditions in order to define their sensitivity, specificity and accuracy.Results: At the end of the third round, a consensus was reached in 139/210 patients (66.2%). The patients with a consensus >= 80% were classified as confirmed BD (n = 24), and those with an agreement of 60-79% as probable BD (n = 10). When comparing these patients with the confounding diseases group, an older age at disease onset, the presence of oral and genital ulcers, skin papulo-pustular lesions, a positive pathergy test and posterior uveitis were BD distinctive elements. The ISG, ICBD and PEDBD criteria were applied to confirmed BD and to the confounding disease group, showing a sensitivity of 0.50, 0.79 and 0.58, a specificity of 1.00, 0.97 and 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively.Conclusions: The PEDBD criteria were very specific, while the ICBD were more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria
Pediatric Inflammatory Multisystem Syndrome and Rheumatic Diseases During SARS-CoV-2 Pandemic
No full textInternational audienceGlobally, the coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), appeared to have a milder clinical course in children compared to adults. As severe forms of COVID-19 in adults included an aberrant systemic immune response, children with chronic systemic inflammatory diseases were cautiously followed. No evidence for a specific susceptibility was identified in this pediatric population. European and US Pediatricians started to notice cases of myocarditis, sharing some features with toxic shock syndrome, Kawasaki disease, and macrophage activation syndrome in otherwise healthy patients. Multisystem Inflammatory Syndrome in Children (MIS-C) and Pediatric Inflammatory Multisystem Syndrome (PIMS) have designated this new entity in the US and Europe, respectively. The spectrum of severity ranged from standard hospitalization to pediatric intensive care unit management. Most patients had a clinical history of exposure to COVID-19 patients and/or SARS-COV2 biological diagnosis. Clinical presentations include fever, cardiac involvement, gastro-intestinal symptoms, mucocutaneous manifestations, hematological features, or other organ dysfunctions. The temporal association between the pandemic peaks and outbreaks of PIMS seems to be in favor of a post-infectious, immune-mediated mechanism. Thus, SARS-CoV2 can rarely be associated with severe systemic inflammatory manifestations in previously healthy children differently from adults highlighting the specific need for COVID-19 research in the pediatric population
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe 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
Full text link“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
Full text linkWe 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
Full text linkWe 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
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