1,721,071 research outputs found
Inside out the thrombus: Defining the role of von Willebrand factor
No full textInside out the thrombus: Defining the role of von Willebrand factor
Pharmacotherapy of von Willebrand disease
No full textIntroduction: 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
No full textRecurrent 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?
No full textThanks 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
Letter by Lippi and Franchini Regarding Article, "ABO Blood Group and Risk of Thromboembolic and Arterial Disease: A Study of 1.5 Million Blood Donors"
No full textABO Blood Group and Risk of Thromboembolic and Arterial Disease: A Study of 1.5 Million Blood Donor
Relative Risks of Thrombosis and Bleeding in Different ABO Blood Groups
No full textThe 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
No full textAlthough 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
No full textComment on Experimental melanoma metastasis in lungs of mice with congenital coagulation disorders. [J Cell Mol Med. 2008
Venous thromboembolism and coffee: critical review and meta-analysis
No full textBACKGROUND: Among the various risk factors of venous thromboembolism (VTE), nutrients seem to play a significant role in the pathogenesis of this condition. This study aimed to clarify the relationship between coffee intake and venous thrombosis, and we performed a critical review of clinical studies that have been published so far.METHODS: An electronic search was carried out in Medline, Scopus and ISI Web of Science with the keywords "coffee" AND "venous thromboembolism" OR "deep vein thrombosis" OR "pulmonary embolism" in "Title/Abstract/Keywords", with no language and date restriction.RESULTS: According to our criteria, three studies (two prospective and one case-control) were finally selected (inter-study heterogeneity: 78%; P<0.001). Cumulative data suggests that a modest intake of coffee (i.e., 1-4 cups/day) may be associated with an 11% increased risk of VTE compared to abstainers, whereas a larger intake (i.e., ≥5 coffee/day) may be associated with a 25% decreased risk.CONCLUSIONS: Our analysis of published data seemingly confirm the existence of a U-shape relationship between coffee intake and VTE, thus exhibiting a trend that overlaps with that previously reported for cardiovascular disease (CVD)
Sleep apnea and venous thromboembolism. A systematic review
No full textRecent evidence suggests that obstructive sleep apnea is a significant and independent risk factor for a number of cardiovascular disorders. Since the association between obstructive sleep apnea and cardiovascular disease is mediated by endothelial dysfunction, hypercoagulability and platelet abnormalities, we sought to investigate whether sleep apnea may also be considered a risk factor for venous thromboembolism (VTE). We carried out an electronic search in Medline and Scopus using the keywords "apnea" OR "apnoea" AND "venous thromboembolism" OR "deep vein thrombosis" OR "pulmonary embolism" in "Title/Abstract/Keywords", with no language or date restriction. Fifteen studies (8 case-control, 4 retrospective observational, 2 prospective case-control and 1 prospective observational) were finally selected for this systematic review. In all studies except one (14/15; 93 %), obstructive sleep apnea was found to be an independent risk factor for VTE, either deep-vein thrombosis (DVT) or pulmonary embolism (PE). In the two prospective case-control studies the risk of DVT or PE was found to be two- to three-fold higher in patients with obstructive sleep apnea than in those without. In conclusion, the current epidemiological evidence supports the hypothesis that obstructive sleep apnea may be an independent risk factor for VTE
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