Results 41 to 50 of about 151,232 (337)

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.
F. Peyvandi, F. Peyvandi, A. Mamaev, J.‐D. Wang, O. Stasyshyn, M. Timofeeva, Nicola Curry, Ana Rosa Cid, T. Yee, K. Kavaklı, G. Castaman, A. Sytkowski   +11 more
semanticscholar   +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

Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease

Case Reports in Hematology, 2015
von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo).
Victoria Campbell, Kevin Marriott, Rex Stanbridge, Abdul Shlebak   +3 more
doaj   +1 more source

Analysis of current perioperative management with Haemate® P/Humate P® in von Willebrand disease: Identifying the need for personalized treatment

Haemophilia, 2018
Patients with Von Willebrand disease (VWD) are regularly treated with VWF‐containing concentrates in case of acute bleeding, trauma and dental or surgical procedures.
H. Hazendonk, J. Heijdra, N. C. D. de Jager, H. Veerman, J. Boender, I. van Moort, R. Mathôt, K. Meijer, B. L. Laros-van Gorkom, J. Eikenboom, K. Fijnvandraat, F. Leebeek, M. Cnossen   +12 more
semanticscholar   +1 more source

DDX3X induces mesenchymal transition of endothelial cells by disrupting BMPR2 signaling

FEBS Open Bio, EarlyView.
Elevated DDX3X expression led to downregulation of BMPR2, a key regulator of endothelial homeostasis and function. Our co‐immunoprecipitation assays further demonstrated a molecular interaction between DDX3X and BMPR2. Notably, DDX3X promoted lysosomal degradation of BMPR2, thereby impairing its downstream signaling and facilitating endothelial‐to ...
Yu Zhang, Jing Wang, De‐Hui Qian, Yang‐Fan Lv, Tian‐Le Cheng, Da‐Peng Wang, Jing Zhang, Ye Fan   +7 more
wiley   +1 more source

Plasma‐Polymerized Nanoparticles Presenting Fibrillin‐1 Drive Rapid Re‐Endothelialization of Vascular Grafts

Advanced Healthcare Materials, EarlyView.
Commercial vascular grafts are made from ePTFE, a highly hydrophobic, foreign material that fails at a high rate in small‐diameter applications. Plasma polymer nanoparticles (PPN) are a versatile material functionalisation tool, used here to present fibrillin‐1 fragment PF8 on the graft surface.
Bob S. L. Lee, Yuen Ting Lam, Alex H. P. Chan, Praveesuda L. Michael, Timothy C. Mitchell, Jui Chien Hung, Miguel Santos, Khoon S. Lim, Richard P. Tan, Steven G. Wise   +9 more
wiley   +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

Multi‐scale Engineered Vasculature and Hierarchical Porosity via Volumetric Bioprinting‐Guided Photopolymerization‐Induced Phase Separation

Advanced Materials, EarlyView.
Vascularization in bioprinted hydrogels is limited by nanoscale porosity in hydrogel bionks. Volumetric bioprinting of gelatin–norbornene phase‐separating hydrogels permits to create centimeter‐scale constructs featuring interconnected multi‐scale porosity. Light dose gradients result in spatially controlled pore dimensions. Capillary‐scale vessels are
Oksana Y. Dudaryeva, Maj‐Britt Buchholz, Gabriel Größbacher, Sofia Amaral, Sammy Florczak, Camille Bonhomme, Alvaro Rojo Ferrer, Mark W. Tibbitt, Riccardo Levato   +8 more
wiley   +1 more source

Biocatalytic Nanoregulators Restore Joint Redox‐Immune Homeostasis in Rheumatoid Arthritis

Advanced Science, EarlyView.
Mesenchymal stem cell‐derived extracellular vesicles (EVs) coat ruthenium‐loaded metal‐organic frameworks (Ru@ZrMOF), creating a targeted therapeutic (Ru@ZrMOF/EVs). This platform scavenges ROS, generates oxygen, and polarizes macrophages from M1 to M2, alleviating inflammation, inhibiting pannus, promoting cartilage repair, and downregulating HIF‐1α ...
Xingheng Wang, Jianbo Huang, Shuwei Zhang, Sujiao Cao, Fangxue Du, Liqiang Zhou, Li Qiu, Yuanjiao Tang   +7 more
wiley   +1 more source