Results 21 to 30 of about 219,337 (367)

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

Emergence of highly profibrotic and proinflammatory Lrat+Fbln2+ HSC subpopulation in alcoholic hepatitis

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, Reika Fujiwara, Stephanie Q. Pan, Khairat B. El‐Baradie, Hye Yeon Choi, Sonal Sinha, Qihong Yang, Kinji Asahina, Yibu Chen, Meng Li, Matthew Salomon, Stanley W.‐K. Ng, Hidekazu Tsukamoto   +12 more
wiley   +1 more source

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, Anne Goodeve, Jeroen Eikenboom   +2 more
doaj   +1 more source

The Molecular Genetics of von Willebrand Disease

Turkish Journal of Hematology, 2012
Quantitative and/or qualitative deficiency of von Willebrand factor (vWF) is associated with the most common inherited bleeding disease von Willebrand disease (vWD). vWD is a complex disease with clinical and genetic heterogeneity.
Ergül Berber
doaj   +1 more source

Factor VIII/von Willebrand factor binding to von Willebrand's disease platelets [PDF]

Blood, 1982
A form of von Willebrand's disease has been described with enhanced ristocetin-induced platelet aggregation and anodal migration of the factor VIII/von Willebrand factor protein (type IIb). We studied two families with this form of von Willebrand's disease and macrothrombocytopenia.
H R, Gralnick, S B, Williams, B C, Shafer, L, Corash   +3 more
openaire   +3 more sources

Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: Relationship with platelet count [PDF]

, 1994
We present a 4-year follow-up of a 42-year-old patient with primary thrombocythemia whose clinical course was complicated by two major mucocutaneous bleeding episodes.
Genderen, P.J.J. (Perry) van, Michiels, J.J. (Jan), Poel-van de Luytgaarde, S.C. (Sonja) van der, Vliet, H.H.D.M. (Huib) van   +3 more
core   +1 more source

VWF/ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma

BMC Gastroenterology, 2019
Background To investigate the von Willebrand factor to ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma (HCC) in cirrhosis.
Hiroaki Takaya, Tadashi Namisaki, Mitsuteru Kitade, Kosuke Kaji, Keisuke Nakanishi, Yuki Tsuji, Naotaka Shimozato, Kei Moriya, Kenichiro Seki, Yasuhiko Sawada, Soichiro Saikawa, Shinya Sato, Hideto Kawaratani, Takemi Akahane, Ryuichi Noguchi, Masanori Matsumoto, Hitoshi Yoshiji   +16 more
doaj   +1 more source

von Willebrand factor regulation of blood vessel formation.

Blood, 2018
Several important physiological processes, from permeability to inflammation to hemostasis, take place at the vessel wall and are regulated by endothelial cells (ECs). Thus, proteins that have been identified as regulators of one process are increasingly
A. Randi, Koval E Smith, G. Castaman
semanticscholar   +1 more source

The influence of protein adsorption on interactions of cultured human endothelial cells with polymers [PDF]

, 1987
A systematic study of the effects of polymer surface properties on the interaction with human endothelial cells (HEC) may lead to the development of small-diameter vascular grafts.
Aken, W.G. van, Bantjes, A., Beugeling, T., Detmers, J.P., Feijen, J., Vreriks, C.M., Wachem, P.B. van   +6 more
core   +9 more sources

A thrombopoietin receptor agonist to rescue an unusual platelet transfusion-induced reaction in a p.V1316M-associated von Willebrand disease type 2B patient

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, Remi Favier, Paulette Legendre, Alexandre Kauskot, Frederic Adam, Veronique Picard, Peter J. Lenting, Cecile V. Denis, Valerie Proulle   +8 more
doaj   +1 more source