Results 21 to 30 of about 151,232 (337)

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

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

Pregnancy and delivery in women with von Willebrand’s disease and different von Willebrand factor mutations

Haematologica, 2010
Background Pregnancy in von Willebrand’s disease may carry a significant risk of bleeding. Information on changes in factor VIII and von Willebrand factor and pregnancy outcome in relation to von Willebrand factor gene mutations are very scanty.Design ...
Giancarlo Castaman, Alberto Tosetto, Francesco Rodeghiero   +2 more
doaj   +1 more source

Insights into pathological mechanisms of missense mutations in C-terminal domains of von Willebrand factor causing qualitative or quantitative von Willebrand disease

Haematologica, 2013
The carboxyl-terminal domains of von Willebrand factor, D4-CK, are cysteine-rich implying that they are structurally important. In this study we characterized the impact of five cysteine missense mutations residing in D4-CK domains on the conformation ...
Hamideh Yadegari, Julia Driesen, Anna Pavlova, Arijit Biswas, Vytautas Ivaskevicius, Robert Klamroth, Johannes Oldenburg   +6 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

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

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

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

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