Results 31 to 40 of about 157,236 (308)

Von Willebrand Disease

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
semanticscholar   +1 more source

Biogenesis of Weibel-Palade bodies in von Willebrand’s disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation

Haematologica, 2012
Background Mutations of cysteine residues in von Willebrand factor are known to reduce the storage and secretion of this factor, thus leading to reduced antigen levels. However, one cysteine mutation, p.Cys2773Ser, has been found in patients with type 2A(
Jiong-Wei Wang, Dafna J. Groeneveld, Guy Cosemans, Richard J. Dirven, Karine M. Valentijn, Jan Voorberg, Pieter H. Reitsma, Jeroen Eikenboom   +7 more
doaj   +1 more source

PREGNANCY AND DELIVERY IN WOMEN WITH VON WILLEBRAND DISEASE

European Journal of Haematology, 2019
Given the wide heterogeneity of phenotypes and of the underlying pathophysiological mechanisms associated with the disorder, pregnancy and delivery in von Willebrand disease (VWD) represent a significant clinical challenge.
G. Castaman, P. James
semanticscholar   +1 more source

Female case with misdiagnosis of hemophilia A who underwent total knee arthroplasty: A case report

Clinical Case Reports, 2022
A female was diagnosed with hemophilia A. She had undergone bilateral total knee arthroplasty. She had a history of numerous hemorrhages including hemarthrosis.
Alireza Bari, Hassan Mansouritorghabeh
doaj   +1 more source

Evolution of replacement therapy for von Willebrand disease: From plasma fraction to recombinant von Willebrand factor.

Blood reviews, 2019
The diagnosis and treatment of von Willebrand disease (VWD) are challenging, in part because patients exhibit a wide range of bleeding patterns and manifestations (e.g.
F. Peyvandi, P. Kouides, P. Turecek, E. Dow, E. Berntorp   +4 more
semanticscholar   +1 more source

Spontaneous iliopsoas muscle hematoma in a patient with von Willebrand disease: a case report

Journal of Medical Case Reports, 2011
Introduction Iliopsoas hemorrhage is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and less commonly in patients with von Willebrand disease.
Soltani Shirazi Ahmad, Keikhaei Bijan
doaj   +1 more source

Efficacy of emicizumab in a pediatric patient with type 3 von Willebrand disease and alloantibodies.

Blood Advances, 2019
Type 3 von Willebrand disease with alloantibodies is a rare clinical entity with few treatment options. Emicizumab prophylaxis in such patients may result in improved hemarthrosis control, lower cost, and enhanced quality of life.
A. Weyand, Veronica H. Flood, J. Shavit, S. Pipe   +3 more
semanticscholar   +1 more source

Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage

Frontiers in Pediatrics, 2022
BackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD).
Songmi Wang, Qun Hu, Yaxian Chen, Xiufen Hu, Ning Tang, Ai Zhang, Aiguo Liu   +6 more
doaj   +1 more source

New therapies for von Willebrand disease.

Blood Advances, 2019
The management of von Willebrand disease (VWD) is based upon the dual correction of the primary hemostasis defect, due to the inherited deficiency of von Willebrand factor (VWF), and of the secondary defect of factor VIII coagulant activity (FVIII:C ...
P. Mannucci
semanticscholar   +1 more source

Health-related quality of life in adults with von Willebrand disease: results of the French real-life Willebrand study on health-related quality of life

Research and Practice in Thrombosis and Haemostasis
Background: Hemorrhagic events in von Willebrand disease (VWD) impair patients’ physical health, daily functioning, and psychological/emotional well-being.
Annie Borel-Derlon, Agnès Veyradier, Yohann Repessé, Nathalie Itzhar-Baïkan, Dominique Desprez, Fabienne Genre-Volot, Hervé Chambost, Laurent Ardillon, Brigitte Pan-Petesch, Sophie Bayart, Malika Barthez-Toullec, Grégory Marin, Marie Hélène André-Bonnet, Sophie Susen, Sylvia von Mackensen, Jenny Goudemand   +15 more
doaj   +1 more source