655 research outputs found

    Inside out the thrombus: Defining the role of von Willebrand factor

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    Inside out the thrombus: Defining the role of von Willebrand factor

    Pharmacotherapy of von Willebrand disease

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    Introduction: Von Willebrand disease (VWD), the most common inherited bleeding disorder, is typically due to quantitative or qualitative deficiencies of von Willebrand factor (VWF).Areas covered: This review, which also summarizes the clinical, pathogenic and diagnostic features of VWD, primarily covers the management of this disorder, focusing on standard and innovative therapies, as well as on different approaches of administration.Expert opinion: The aim of treatment is to correct the dual defect of hemostasis caused by abnormal/reduced VWF and concomitant deficiency of factor VIII (FVIII). The therapy is indicated for management of bleeding and for short-term (i.e., surgical or invasive procedure) or long-term prophylaxis. The synthetic vasopressin analogue desmopressin (DDAVP) is the treatment of choice for about 80% of VWD patients, whereas replacement therapy with FVIII/VWF concentrates is indicated for those who are unresponsive to DDAVP

    Thalidomide for hereditary haemorrhagic telangiectasia

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    Recurrent and severe epistaxis is the most common presentation of hereditary haemorrhagic telangiectasia, a rare autosomal dominant bleeding disorder characterised by mucocutaneous telangectasias and arteriovenous malformations.1,2 The onset of this severe symptom can lead to anaemia requiring frequent intravenous infusions of iron and, often, transfusion of red blood cells. Several approaches have been attempted to manage the nosebleeds of patients with hereditary haemorrhagic telangiectasia, including compression techniques, bilateral embolisation, and surgical arterial ligation, in addition to other, less commonly applied, treatments, such as laser therapy, sclerosing agents, electrocauterisation, and septodermoplasty

    Prevention of inhibitor development in hemophilia A in 2016. A glimpse into the future?

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    Thanks to considerable progresses made over the last 30years, hemophilia benefits from the most efficacious and safe treatment among the many monogenic inherited disorders. The most challenging complication of replacement therapy in hemophilia A is the occurrence of alloantibodies against infused factor VIII (FVIII), thus predisposing the patients to increased morbidity and disability. Extensive research in this field has definitively unraveled that development of inhibitors in hemophilia A is a complex and multifactorial process, in which inherited and environmental factors dynamically interact. This narrative review, after providing a concise overview about the main genetic and non-genetic risk factors, is aimed to focus on prediction risk models and preventive strategies for minimizing the risk of developing inhibitors in hemophilia A patients

    Relative Risks of Thrombosis and Bleeding in Different ABO Blood Groups

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    The ABO blood group system is composed of complex carbohydrate molecules (i.e., the A, B, and H determinants) that are widely expressed on the surface of red blood cells and in a variety of other cell and tissues. Along with their pivotal role in transfusion and transplantation medicine, the ABO antigens participate in many other physiological processes and, in particular, are important determinants of von Willebrand factor and factor VIII circulating plasma levels. The precise influence of the ABO system on hemostasis has led the way to the investigation of a putative implication in the risk of developing cardiovascular disorders. Along with the underlying molecular mechanisms, the current knowledge on the role of ABO blood group antigens in both the thrombotic and hemorrhagic risk will be summarized in this narrative review

    Platelets and immunity: the interplay of mean platelet volume in health and disease

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    Although it is still unclear whether larger platelets actively participates to the pathogenesis of human diseases or their appearance represents a simple epiphenomenon of an underlying derangement of platelet biology, several lines of evidence seemingly attest that these blood elements may play an active role in certain pathologies, such as myocardial infarction, venous thromboembolism, cancer, and severe infections. The mean platelet volume is a simple measure of platelet size, which is automatically generated by virtually all modern hematologic analyzers. Its measurement should hence be regarded as a valuable perspective of clinical outcome prediction

    Are haemophiliacs protected against cancer development? Prospective controlled studies are needed

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    Comment on Experimental melanoma metastasis in lungs of mice with congenital coagulation disorders. [J Cell Mol Med. 2008

    O Araguaia pelos Militares: Imaginários e Barbáries

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    Review text “Araguaia by the Military: Imaginary and Barbaries” (2008), by the author Flávia M. Franchini Ribeiro.Review text “Araguaia by the Military: Imaginary and Barbaries” (2008), by the author Flávia M. Franchini Ribeiro.Texto da resenha "O Araguaia pelos Militares: Imaginários e Barbáries " da autora Flávia M. Franchini Ribeiro

    Gianni Franchini (1934-2010)

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