Results 21 to 30 of about 44,755 (204)

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

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

A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF/FVIII concentrates in severe inherited von Willebrand disease [PDF]

, 2019
BACKGROUND: There is a lack of prospective clinical trials specifically designed to evaluate the benefits of prophylaxis with vWF/FVIII concentrates in patients with inherited von Willebrand disease (vWD).
De Cristofaro, Raimondo
core   +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

von Willebrand Disease

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 ...
W C, Nichols, D, Ginsburg
openaire   +2 more sources

Von Willebrand disease: gaining a global perspective [PDF]

, 2023
Introduction Recent guidelines for von Willebrand Disease (VWD) highlighted the challenges in diagnosis and management. Identifying the number of persons with VWD (PwVWD) internationally will help target support to aid diagnosis of PwVWD.
Rotellini, D., Makris, M., Lavin, M., Alfonso Iorio, Ziemele, B., Tootoonchian, E., Glenn F. Pierce, Claudia Khayat Djambas, Khayat Djambas, C., Iorio, A., Robert F. Sidonio, Federici, A.B., Stonebraker, J., Dawn Rotellini, Magdy El Ekiaby, O'Sullivan, J.M., Jeffrey Stonebraker, Baiba Ziemele, Jamie M. O'Sullivan, Coffin, D., Michael Makris, El Ekiaby, M., Pierce, G.F., Ellia Tootoonchian, Augusto B. Federici, Sidonio, R.F., Donna Coffin, James, P.D., Michelle Lavin, Paula D. James   +29 more
core   +3 more sources

Closing the gap – detection of clinically relevant von Willebrand disease in emergency settings through an improved algorithm based on rotational Thromboelastometry

BMC Anesthesiology, 2019
Background Hemorrhage and blood loss are still among the main causes of preventable death. Global hemostatic assays are useful point-of-care test (POCT) devices to rapidly detect cumulative effects of plasma factors and platelets on coagulation ...
H.-G. Topf, E. R. Strasser, G. Breuer, W. Rascher, M. Rauh, F. B. Fahlbusch   +5 more
doaj   +1 more source

Doença de von Willebrand e anestesia Enfermedad de von Willebrand y anestesia Von Willebrand's disease and anesthesia

Revista Brasileira de Anestesiologia, 2007
JUSTIFICATIVA E OBJETIVOS: A doença de von Willebrand ocorre devido à mutação no cromossomo 12 e é caracterizada por deficiência qualitativa ou quantitativa do fator de von Willebrand.
Fabiano Timbó Barbosa, Rafael Martins da Cunha, Luciano Timbó Barbosa   +2 more
doaj   +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

Platelet-independent adhesion of calcium-loaded erythrocytes to von Willebrand factor. [PDF]

PLoS ONE, 2017
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage.
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk   +7 more
doaj   +1 more source