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Medicine, 1997 Considerable progress has been made in characterizing the specific molecular defects responsible for the heterogeneous disorder known as von Willebrand disease (VWD). A large number of molecular defects have been identified and precise characterization may now be possible in the majority of type 2A, type 2B, type 2N, and potentially also type 3 VWD ...
William C. Nichols, David Ginsburg
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von Willebrand's disease [PDF]
European Journal of Haematology, 1975 The common inheritable hemorrhagic disorder, von Willebrand's disease, has been known for only about 50 yr. During these years it has been well established that the cause of the disease is a deficiency of a plasma protein with remarkable biological properties. Future research should focus on details of the molecular structure of this protein.
Lars Holmberg, Inga Marie Nilsson
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Hematology, 2014 Von Willebrand disease (VWD) is an inherited defect of blood coagulation that may cause excessive bleeding.
Stacy Cooper, Clifford M. Takemoto
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Genetics in Medicine, 2011 von Willebrand disease is a common inherited bleeding disorder characterized by excessive mucocutaneous bleeding. Characteristic bleeding symptoms include epistaxis, easy bruising, oral cavity bleeding, menorrhagia, bleeding after dental extraction, surgery, and/or childbirth, and in severe cases, bleeding into joints and soft tissues.
Paula D. James, Anne Goodeve
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Treatment of Von Willebrand Disease [PDF]
Thrombosis and Haemostasis, 2001 SummaryIn von Willebrand disease, there are two main options for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1, who account for approximately 70 to 80 per cent of all cases with the disease ...
P M Mannucci
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Hepatology, EarlyView., 2022 Lrat+ quiescent hepatic stellate cells (qHSC) give rise to Lrat+Fbln2+ activated HSC (aHSC) in alcohol‐associated hepatitis and this subpopulation is highly profibrotic, inflammatory, and immunoregulatory based on their single cell transcriptomic profile. Abstract Background and Aims Relative roles of HSCs and portal fibroblasts in alcoholic hepatitis (
Steven Balog +12 more
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Therapeutic Advances in Hematology, 2022 This report describes the first case of splenic injury in a patient with p.V1316M-associated von Willebrand disease type 2B (VWD2B) with chronic thrombocytopenia, successfully treated with nonoperative management including von Willebrand factor (VWF ...
Caterina Casari +8 more
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Haematologica, 2011 Background Nucleotide variations not changing protein sequences are considered silent mutations; accumulating data suggest that they can, however, be important in human diseases.Design and Methods We report an altered splicing process induced by a silent
Viviana Daidone +6 more
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Principles of care for the diagnosis and treatment of von Willebrand disease
Haematologica, 2013 Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in ...
Giancarlo Castaman +2 more
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Handbook of Pediatric Hematology and Oncology, 2016 von Willebrand disease (VWD), the most common inherited bleeding disorder, is caused by quantitative or qualitative deficiencies in the von Willebrand factor (VWF) protein.
Trinh T Nguyen, Lakshmi V. Srivaths
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