File Name: von willebrand disease basic and clinical aspects .zip
Information regarding the status of von Willebrand disease VWD in Spain was very scarce until , when a survey related to the referred patients to each center was carried out with a good rate of response
Blood ; 6 : — Type 2 von Willebrand disease VWD includes a wide range of qualitative abnormalities of von Willebrand factor structure and function resulting in a variable bleeding tendency. According to the current classification, 4 different subtypes can be identified, each with distinctive phenotypic and therapeutic characteristics.
Subtyping von Willebrand disease VWD as type 1 most common , type 2 variants less common , or type 3 rare. This test is not useful for detection of hemophilia carriers. If any test results in the profile are abnormal, all results will be reviewed by a coagulation consultant and a von Willebrand Disease Profile Interpretation AVWPQ will be provided. VWF serves as an adhesive protein important in adhering platelets to subendothelial tissue at the site of vascular injury and for adhering platelets to each other aggregation. Platelet adhesion and aggregation are essential to form a mechanical hemostatic "plug" and as the focus for interaction of clotting factors and phospholipid required for the formation of the fibrin platelet clot.
Patient information: See related handout on von Willebrand disease , written by the authors of this article. Von Willebrand disease is an inherited condition characterized by deficiency of von Willebrand factor, which is essential in hemostasis. The National Heart, Lung, and Blood Institute has released new evidence-based guidelines for the diagnosis and management of the disease. There are three major subtypes of von Willebrand disease, classified as partial quantitative deficiency low levels of von Willebrand factor type 1 , qualitative deficiency type 2 , or virtually complete deficiency type 3. Diagnosis is usually made by reviewing the patient's personal and family history of bleeding and by clinical evaluation for more common reasons for bleeding, supplemented with laboratory tests. Assessment may be used to determine bleeding risk before surgery and other invasive procedures, and to diagnose reasons for unexplained hemorrhaging. Von Willebrand factor levels of 30 IU per dL or lower are required for the definite diagnosis of inherited von Willebrand disease.
Autoimmune paraphenomena, are associated with B-cell lymphoproliferative disorders, including monoclonal gammopathy of uncertain significance. These paraphenomena can rarely include acquired bleeding disorders. Acquired bleeding disorders are often underdiagnosed and a high degree of clinical suspicion is required. There was an absence of platelet aggregation to all tested agonists including ristocetin. Once the diagnosis was made, however, the patient showed a partial response to intravenous immunoglobulin confirming the immunological pathogenesis in this case. This case highlights the need to consider acquired bleeding disorders in patients with a possible predisposing factor. MGUS is associated with an increase in auto-immune paraphenomena including haematological disorders.
The development of registries through international collaboration has facilitated better understanding of the rare bleeding disorders. Such work has shown that rare bleeding disorders are heterogeneous and need to be studied singularly, and that heterozygous patients may bleed. There is a need to understand the minimum plasma coagulant activity level to prevent spontaneous bleeding. Moreover, due to the low prevalence of rare bleeding disorders, the management of this patient population remains a challenge. Data collection on clinical history, efficacy and side effects of treatment needs to be harmonised. Rare diseases have a low prevalence and affect a small number of people.
Von Willebrand Disease: Basic and Clinical Aspects provides an insight into all aspects of the condition. Since its discovery, von Willebrand disease has been extensively studied and the causative factor deficiency, the understanding of the condition and its treatment has greatly improved. This book summarizes recent research and will help to optimize the management of patients with von Willebrand disease. This valuable book describes the important and complex role of von Willebrand factor in hemostasis and thrombosis. In addition to the current understanding of its molecular biology, this book gives particular focus to the association between genetic variants of von Willebrand factor and different von Willebrand disease phenotypes.
Von Willebrand Disease: Basic and Clinical Aspects is a valuable resource for hematologists in practice and in training, and specialists in thrombosis and.
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Acquired von Willebrand syndrome AvWS is a rare but probably underestimated bleeding disorder characterized by laboratory findings and clinical presentations similar to those of inherited von Willebrand disease vWD. Although it was first recognized more than 50 years ago it was described in in a patient with systemic lupus erythematosus , AvWS has gained renewed interest in the last few years due to its association with relatively frequent cardiovascular disorders, including congenital heart defects, aortic stenosis, and the use of left ventricular assist devices. This article reviews current knowledge on the mechanisms, diagnostic, clinical and therapeutic aspects of AvWS, focusing particularly on those cases associated with hematologic disorders.
Beyond its role in hemostasis, von Willebrand factor VWF is an emerging mediator of vascular inflammation. Moreover, VWF and ADAMTS13 have been recently proposed as prognostic biomarkers in cardiovascular, metabolic, and inflammatory diseases, such as diabetes, stroke, myocardial infarction, and sepsis. All these features make VWF an attractive therapeutic target in thromboinflammation. Results from animal models and clinical studies support the potent anti-inflammatory and antithrombotic effect of VWF antagonism, providing reassuring data on its safety profile. The story of von Willebrand factor starts in Finland in the first years of the s.
Передо мной лежит отчет, из которого следует, что ТРАНСТЕКСТ бьется над каким-то файлом уже восемнадцать часов и до сих пор не вскрыл шифр. Джабба обильно полил приправой кусок пирога на тарелке. - Что-что.
С какой стати университетский профессор… Это не университетские дела. Я позвоню и все объясню. Мне в самом деле пора идти, они связи, обещаю. - Дэвид! - крикнула .
Капля Росы. Что-то в этом абсурдном имени тревожно сверлило его мозг. Капля Росы. Он слышал приятный голос сеньора Ролдана из агентства сопровождения Белена.
Тебе он всегда рад. Сьюзан заставила себя промолчать. Хейл хмыкнул себе под нос и убрал упаковку тофу. Затем взял бутылку оливкового масла и прямо из горлышка отпил несколько глотков. Он считал себя большим знатоком всего, что способствовало укреплению здоровья, и утверждал, что оливковое масло очищает кишечник.
Сьюзан пыталась отстраниться, но он не отпускал. ТРАНСТЕКСТ задрожал, как ракета перед стартом. Шифровалка содрогалась. Стратмор сжимал ее все сильнее. - Останься со мной, Сьюзан.
Мы идем ко дну. ГЛАВА 120 Шеф отдела обеспечения системной безопасности, тучный мужчина весом за центнер, стоял неподвижно, заложив руки за голову. Он не мог поверить, что дожил до подобной катастрофы.
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