1,721,132 research outputs found
Thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome (congenital and acquired)
No full textRare bleeding disorders
No full textF5F8D, and mutations -in LMAN1 or MCFD2 proteins; Factor V deficiency -FV, double role in coagulation process; Factor X deficiency -FX glycoprotein, pivotal role in coagulation cascade; Genetic variants -identified along prothrombin gene; Knockout mouse models -coagulation factors and vitamin K dependent proteins; Patients with FXIII-A deficiency -bleeding tendency, life-threatening symptoms as umbilical-cord and CNS bleeding; Prothrombin deficiency -rarest inherited coagulation disorder; Rare bleeding disorders; Rare bleeding disorders (RBDs), inherited deficiencies -coagulation factors as fibrinogen, factor (F) II, FV, combined FV and FVIII; Vitamin K-dependent coagulation factors deficiency (VKCFD
Treatment of T-cell large granular lymphocytic leukaemia the role for methotrexate and cyclosporin A in new, relapsed and refractory cases.
No full textThrombotic thrombocytopenic purpura and haemolytic uraemic syndrome (congenital and acquired)
No full textThe efficacy of alemtuzumab for refractory chronic lymphocytic leukemia in relation to cytogenetic abnormalities of p53
No full textWe reviewed the efficacy of alemtuzumab in the treatment of 28 patients with refractory chronic lymphocytic leukemia (CLL) in whom p53 status was known. Overall responses of 53.6% (complete responses 17.9%) were attained with no significant difference between patients with (50%) or without (55%) p53 deletion (p=0.214). We confirm the efficacy of alemtuzumab in refractory CLL irrespective of p53 deletions, and advocate its introduction earlier in disease course
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
T gamma cell deficiency in idiopathic thrombocytopenic purpura (ITP).
No full textA significant reduction in the proportion and absolute number of circulating T gamma (suppressor) lymphocytes was observed in 15 adult patients with idiopathic thrombocytopenic purpura (ITP). The proportion of T gamma cells was also reduced in the spleen of four patients so investigated. This abnormality was not seen in 5 patients cured by splenectomy whilst it persisted in 3 who remained thrombocytopenic after splenectomy. The proportion of Tmu (helper) lymphocytes in ITP was normal. These findings, similar to those reported in systemic lupus erythematosus, suggest that an inbalance of the immunoregulatory T-cell subsets may be important in the pathogenesis of ITP
Prolonged treatment response in aggressive natural killer cell leukemia
No full textWe describe a case of natural killer (NK) cell leukemia with acute presentation, systemic symptoms and hepatosplenomegaly. The uniform and aberrant phenotype of NK cells with infiltration of bone marrow and spleen was in keeping with a malignant diagnosis. Aggressive presentation was demonstrated by marked constitutional symptoms and significant tumor burden (liver, spleen, blood, bone marrow). The subsequent clinical course has been indolent, but this may have been influenced by treatment. Treatment consisted sequentially of splenectomy, intravenous pentostatin and the combination of cyclosporine A and recombinant human erythropoietin and has resulted in survival of over 48 months. We discuss the difficulties in the diagnosis of this condition, explore possible causes of cytopenia(s), and highlight the role of immunosuppression in controlling disease manifestations in large granular lymphocyte proliferative disorders
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
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