1,721,195 research outputs found

    Intracranial haemorrhage in children and adults with haemophilia A and B: A literature review of the last 20 years

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    Intracranial haemorrhage (ICH) is the most serious event in haemophiliacs, resulting in high rates of mortality and disability. Although the use of a prophylaxis regimen has improved outcomes, the mortality caused by ICH is still around 20%. ICH is more frequent at two different ages: in childhood (mostly in children aged ≤2 years) and in adulthood (with known risk factors such as hypertension and age ≥60 years). Our review shows how ICH remains one of the worst problems of patients with haemophilia. Greater attention to risk factors and early symptoms, together with an appropriate early prophylaxis, may reduce the risk of severe intracranial haemorrhagic events

    Savings without changing: How to use the MyPKfit® device to improve treatment strategies in a cohort of patients with haemophilia A

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    Background: The real goal on haemophilia treatment is to combine efficacy, safety, improvement in quality of life and cost-savings. Sometimes the choice for reaching this result is to switch the patients to an extended half-life (EHL) drug. In case of haemophilia A this goal is not always achieved due to the less pharmacokinetic (PK) differences among EHL and standard concentrates. A better and regular use of available tools, as MyPKfit®, can then optimize the treatment without distorting therapy or changing concentrate. Methods: We now report our experience with a population of severe or moderate haemophilia A patients treated with octocog-alfa (Advate® –Shire Takeda) and in which a tailored prophylaxis with MyPKfit® has been assessed. Results: PK evaluations of 14 patients were carried out. A Bayesian curve and a tailored prophylaxis were assessed individually employing PK data. The weekly frequency of infusions was reduced in three severe patients, it was increased in four while it remained the same in the others five patients. The annual consumption of concentrate was reduced in 81.8% of patients. A subsequent economic evaluation carried out for each of the twelve severe haemophilia A patients included in this analysis, in which we have compared the standard and the PK-driven prophylaxis, showed that an optimized treatment can lead to an annual average saving of € 20,525 (−15.8%). Conclusions: The use of MyPKfit® for a tailored prophylaxis may lead to a more rational use of available resources through an easy correction of the treatment strategies without distorting the individual patient therapy

    Immune tolerance induction rescue with turoctocog-alfa in a poor risk haemophilia A inhibitor young child: The history of a success

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    The development of alloantibodies against the replacement of Factor VIII (FVIII) is the major complication in haemophilia A treatment. The gold standard to eradicate inhibitors is the immune tolerance induction (ITI), but in some cases it fails requiring another immune tolerance, defined ITI rescue (ITIR), using a different concentrate, even though it is still debated. We report a successful case of a poor risk (titre of inhibitor at start of ITI>10 BU/ml, peak titre on ITI>200 BU/ml, >2 years since the inhibitor diagnosis) haemophilia A child treated with a high-dose regimen (200 UI/kg/day) turoctocog-alfa after a failed first-line ITI with octocog-alfa lasting 29 months. At 22 months of ITI-R, the inhibitor titre was undetectable, the FVIII recovery was 74%, of the expected level and the FVIII half-life more than 7 h. A complete successful ITI-R was then achieved with turoctocog-alfa

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The 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

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    “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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