Results 31 to 40 of about 146,739 (334)

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

How I manage severe von Willebrand disease

British Journal of Haematology, 2019
Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin.
F. Leebeek, F. Atiq
semanticscholar   +1 more source

Phase 3 study of recombinant von Willebrand factor in patients with severe von Willebrand disease who are undergoing elective surgery

Journal of Thrombosis and Haemostasis, 2018
Essentials Recombinant von Willebrand factor (rVWF) is effective in von Willebrand disease (VWD). A phase 3 study of rVWF, with/without recombinant factor VIII (rFVIII) before surgery in VWD.
Flora Peyvandi, Flora Peyvandi, A. Mamaev, J.‐D. Wang, O. Stasyshyn, M. Timofeeva, Nicola Curry, Ana Rosa Cid, T. Yee, K. Kavakli, G. Castaman, A. Sytkowski   +11 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

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

Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders

Journal of Clinical Medicine, 2017
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom.
G. Castaman, S. Linari
semanticscholar   +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

A comparative analysis of different automated von Willebrand factor glycoprotein Ib‐binding activity assays in well typed von Willebrand disease patients

Journal of Thrombosis and Haemostasis, 2018
Essentials Von Willebrand ristocetin cofactor activity (VWF:RCo) is not a completely reliable assay. Three automated VWF activity assays were compared within a von Willebrand disease (VWD) cohort.
I. Vangenechten, K. Mayger, P. Smejkal, Ondřej Zapletal, J. J. Michiels, Gary W. Moore, A. Gadisseur   +6 more
semanticscholar   +1 more source

Factor fon Willebrand antigen in endothelium functional state assessment in patients with systemic lupus erythematosus

Научно-практическая ревматология, 2004
Objective. To determine the importance of von Willebrand factor antigen as marker of endotheliae damage at the patients with systemic lupus erythematosus (SLE). Material and methods. 30 women from authentic SLE by criteria ACR, in the age of 20-45 years,
M E Zapryagaeva, E S Mach, A A Baranov
doaj   +1 more source

Analysis of von Willebrand Disease in the “Heart of Europe”

TH Open, 2022
Background von Willebrand disease (VWD) is a genetic bleeding disorder caused by defects of von Willebrand factor (VWF), quantitative (type 1 and 3) or qualitative (type 2). The laboratory phenotyping is heterogenic making diagnosis difficult.
Inge Vangenechten, Petr Smejkal, Jiri Zavrelova, Ondrej Zapletal, Alexander Wild, Jan Jacques Michiels, Zwi Berneman, Jan Blatny, Angelika Batorova, Tatiana Prigancova, Miroslav Penka, Alain Gadisseur   +11 more
doaj   +1 more source