Von Willebrand Disease Basic And Clinical Aspects Pdf
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- Diagnosis and management of von willebrand disease in Spain
- The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
- Diagnosis and Management of Von Willebrand Disease: Guidelines for Primary Care
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.
Diagnosis and management of von willebrand disease in Spain
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.
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. VWF also stabilizes plasma factor VIII by binding it and protecting it from proteolysis and serves as a carrier protein for that clotting factor. VWF circulates in the blood in 2 distinct compartments.
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
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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. It also reviews the important area of the obstetric and gynecological manifestations of von Willebrand disease, as well as the treatment of acute bleeding. Written by an international team of contributors it describes the progress in, and difficulties of diagnosing phenotypes and genotypes.
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 Obviously due to the limited use of the ristocetin induced platelet agglutination RIPA assay it was expected that some patients with type 2B and pseudo-VWD were misdiagnosed. Sixty-five percent of the participants never requested this assay, which suggested that some patients with this subtype type 2N VWD could be misdiagnosed as hemophilia A, or underdiagnosed. Six centers had the methodology available to perform a multimeric analysis of VWF; however, one abandoned it in because the test was very cumbersome. Twenty hospitals requested this analysis from an external laboratory, although not for all patients. Seventeen percent of the participants never requested this assay.
Von Willebrand Disease: Basic and Clinical Aspects · Augusto B. Federici MD, · Christine A. Lee MA, MD, DSc (Med), FRCP, FRCPath, FRCOGad.
Diagnosis and Management of Von Willebrand Disease: Guidelines for Primary Care
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.
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. Current available laboratory methods allow a straightforward approach to VWD subtyping, and although the precise molecular characterization remains complex, it is not required for appropriate treatment of the vast majority of cases. Desmopressin can be useful only in a few type 2 cases compared with patients with actual quantitative deficiency type 1 , most often in variants with a nearly normal multimeric pattern type 2M.
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.
Три шкафа-картотеки стояли в углу рядом с маленьким столиком с французской кофеваркой. Над Форт-Мидом высоко в небе сияла луна, и серебристый свет падал в окно, лишь подчеркивая спартанскую меблировку. Что же я делаю. - подумал Бринкерхофф. Мидж подошла к принтеру и, забрав распечатку очередности задач, попыталась просмотреть ее в темноте. - Ничего не вижу, - пожаловалась .
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Их отношения развивались медленно и романтично: встречи украдкой, если позволяли дела, долгие прогулки по университетскому городку, чашечка капуччино у Мерлутти поздно вечером, иногда лекции и концерты. Сьюзан вдруг поняла, что стала смеяться гораздо чаще, чем раньше. Казалось, не было на свете ничего, что Дэвид не мог бы обратить в шутку. Это было радостное избавление от вечного напряжения, связанного с ее служебным положением в АНБ.
Коммандер! - из последних сил позвала Сьюзан. Хейл развернул Сьюзан в ту сторону, откуда слышался голос Стратмора. - Выстрелишь - попадешь в свою драгоценную Сьюзан.