1,720,964 research outputs found
The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy.
No full textHLA-DP GENOTYPING IN PATIENTS WITH SYSTEMIC LUPUS-ERYTHEMATOSUS - CORRELATIONS WITH AUTOANTIBODY SUBSETS
No full textThe antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy
No full textHLA-DP genotyping in patients with systemic lupus erythematosus: correlations with autoantibody subsets
No full textIn order to ascertain whether the HLA-DP locus plays a role in the genetic predisposition for systemic lupus erythematosus (SLE), 42 patients with SLE were typed for 17 different DPBeta haplotypes by locus specific amplification followed by allele specific oligonucleotide hybridization. Sera from the same patients were assayed for the presence of autoantibodies to Sm, RNP, Ro, La and of anticardiolipin antibodies (aCL). DPB1*0301 and DPB1*1401 were increased in patients compared with 107 healthy controls, mainly in those anti-Sm/RNP positive and aCL positive. Remarkably, DPB1*1401 and DPB1*0301 have a nearly identical nucleotide sequence, suggesting that an epitope shared by their membrane products is of major importance for the DP related susceptibility to SLE
Síndrome antifosfolipídico catastrófico
Full text link[spa] INTRODUCCIÓN: El síndrome antifosfolipídico catastrófico fue descrito por Asherson en el año 1992 como una variante del síndrome antifosfolipídico (SAF) que conduce a insuficiencia multiorgánica, caracteriza por la oclusión trombótica de los vasos de pequeño calibre, que se produce en un breve período de tiempo en presencia de anticuerpos antifosfolipídicos. Representa aproximadamente el 1% de los pacientes con SAF, pero la mortalidad es mayor del 50%. Las causas y los factores pronósticos que influyen es esta elevada mortalidad son desconocidos.HIPÓTESIS: El SAF catastrófico debería ser considerado en el diagnóstico diferencial de los pacientes con fallo multiorgánico (FMO), síndrome de distrés respiratorio agudo (SDRA) y anenia hemolítica microangiopática (AHM). El esquema terapéutico combinando glucorticoides (GC) junto a anticoagulación efectiva (AC) y recambio plasmático (RP), sería es el que consigue mayores tasas de supervivencia. Por tanto, la utilización de esta triple terapia podría mejorar la evolución de estos pacientes.OBJETIVOS: Analizar las características clínica y biológicas de los pacientes con SAF catastrófico, especialmente en aquellos que desarrollan SDRA y AHM. Determinar las causas y los factores pronósticos que condicionan su elevada mortalidad como así también la influencia del tratamiento en la evolución de estos enfermos.MATERIAL Y MÉTODOS:Debido a la baja prevalencia del síndrome es imposible que un solo centro reúna un número de pacientes suficientes para realizar estudios de investigación, por lo que se creó un registro internacional denominado "CAPS registry" con el fin de reunir todos los pacientes con SAF catastrófico. El servicio de enfermedades autoimmnes del Hospital Clínico de Barcelona es el centro coordinador para la recepción de la información y los directores y doctorando de esta tesis son los responsables del diseño, confección y actualización del mismo. El registro está patrocinado por el Europeam Forum on Antiphospholipid Antibodies y se diseñó como un portal abierto de libre acceso a través de la siguiente dirección: www.med.ub.es /MIMMUN/FORUM/CAPS. HTM. TRABAJOS PUBLICADOS:1. Espinosa G, Bucciarelli S, Cervera R et al. Thrombotic hemolitic microangiopathic anaemia and antiphospholipid antibodies. Ann Reum Dis 2004; 63: 730-736 (IF 6,96). 46 pacientes con AHM y anticuerpos antifosfolipídicos (aFL) fueron revisados. La presentación clínica fué: síndrome hemolítico urémico (26%), SAF catastrófico (23%), purpura trombótica trombocitopénica (13%) y síndrome HELLP (4%). El 70 % de los pacientes que recibieron recambio plasmático se recuperaron. La mortalidad fué del 22%.2. Bucciarelli S, Espinosa G, Cervera R. The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome.Ann Rheum Dis 2006; 65:81-86 (IF : 6,96). El 68 % de los pacientes tuvieron afectación pulmonar y en el 31% se manifestó como SDRA. El 70% de los pacientes en los que se dispuso de anatomía patológica presentaron microtrombosis.3. Bucciarelli S, Espinosa G, Cervera R et al. Mortality in the catastrophic antiphospholipid syndrome. Prognostic factors in a series of 250 patients. Arthritis Rheum 2006 (en prensa) (IF: 7,42) La mortalidad global fue del 44%. La principal causa de muerte fue la afectación neurológica (27,9%) seguida por la afectación cardiaca y las infecciones en un 19,8%. La presencia de lupus eritematoso sistémico se asoció a una mayor mortalidad. La mayor tasa de recuperación estuvo se obtuvo con la utilización de GC+AC+RP. La mortalidad disminuyó un 20% después del 2001 asociada a una mayor utilización de la terapia combinada con GC+AC+RP.CONCLUSIONES:El SAF catastrófico debe ser considerado en el diagnóstico diferencial de los pacientes con FMO, SDRA y AHM. El tratamiento combinado con AC+GC+RP debe ser considerada como terapia de primera línea en los pacientes con SAF catastrófico.[eng] "CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME"BACKGROUND: The term catastrophic antiphospholipid syndrome (APS) was proposed by Asherson in 1992 for defining an accelerated form of APS resulting in multiorgan failure, developed in a very short period of time. Although patients with catastrophic APS represent less than 1% of patients with APS, the mortality rate is more than 50%. The causes of this high mortality are still unknown.HYPOTHESES: The catastrophic APS would be considered as differential diagnosis among multiorgan dysfunction, thrombotic haemolytic microangiopathic anaemia and acute respiratory distress syndrome. A higher recovery rate would be associated with combined treatment with anticoagulation + corticosteroids + plasma exchange (AC+CS+PE). OBJECTIVES: to analyse the clinical and laboratory features of patients with catastrophic APS mainly in those patients who develop thrombotic miroangiopathic anaemia (THMA) and acute respiratory distress syndrome (ARDS). To assess the main causes of death and the prognostic factors that can influence mortality in patients with catastrophic APS.METHOD: We analysed the patients with catastrophic APS included in the "CAPS registry" in order to achieve the objectives.RESULTS: FIRST STUDY: 46 patients were reviewed with AMTH and aPL. The clinical presentations were: hemolytic-uremic syndrome (26%), catastrophic APS (23%), thrombotic thrombocytopenic purpura (13%), and HELLP syndrome (4%). Recovery occurred in 70% of episodes treated with Plasm Exchange. SECOND STUDY: Pulmonary involvement was reported in 68% patients with catastrophic APS and 31% of them were diagnosed as having ARDS. Microthromboses was present in 70% patients. THIRD STUDY: Death occurred in 44%. Cerebral involvement was considered the main cause of death (27.2%) followed by cardiac involvement and infection (19.8% one each). The presence of systemic lupus erythematosus was associated with a higher mortality. A higher recovery rate was associated with combined treatment with anticoagulation + corticosteroids + plasma exchange (AC+CS+PE) (77.8%). The mortality decreased 20% from 2001 associated with the higher use rate of combined treatment with AC+CS+PE and/or IVIg.CONCLUSIONS: The catastrophic APS should be considered as differential diagnosis among multiorgan dysfunction, thrombotic haemolytic microangiopathic anaemia and acute respiratory distress syndrome. The combined therapy with AC+CS+PE should be the first line of therapy in patients with catastrophic APS
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
- …
