Results 21 to 30 of about 157,236 (308)

New developments in von Willebrand disease

British Journal of Haematology, 2020
Von Willebrand disease (VWD) constitutes the most common inherited human bleeding disorder. It is associated with a mucocutaneous bleeding phenotype that can significantly impact upon quality of life.
H. Fogarty, D. Doherty, James S. O’Donnell   +2 more
semanticscholar   +1 more source

Clinical Spectrum and Laboratory Study of Von Willebrand Disease -Experience from Tertiary Care Hospital in Pakistan

Pakistan Armed Forces Medical Journal, 2022
Objective: To determine the clinical features and laboratory parameters of patients of von Willebrand disease (vWD) in our population. Study Design: Cross-sectional study.
Mohsin Hussain, Asad Mahmood, Rafia Mahmood, Ayesha Khurshid, Nabeela Khan, Nozaif Sarwar   +5 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

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

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

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

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

Bleeding symptoms in patients diagnosed as type 3 von Willebrand disease: Results from 3WINTERS‐IPS, an international and collaborative cross‐sectional study

Journal of Thrombosis and Haemostasis, 2020
Type 3 von Willebrand's disease (VWD) patients present markedly reduced levels of von Willebrand factor and factor VIII. Because of its rarity, the bleeding phenotype of type 3 VWD is poorly described, as compared to type 1 VWD.
A. Tosetto, Zahra Badiee, M. Baghaipour, L. Baronciani, J. Battle, E. Berntorp, I. Bodó, U. Budde, G. Castaman, J. Eikenboom, P. Eshghi, C. Ettorre, A. Goodeve, J. Goudemand, Hay Charles Richard Morris, Hamid Hoorfar, M. Karimi, B. Keikhaei, R. Lassila, F. Leebeek, M. F. López Fernández, P. Mannucci, M. Mazzucconi, M. Morfini, J. Oldenburg, I. Peake, R. Parra López, F. Peyvandi, R. Schneppenheim, A. Tiede, G. Toogeh, M. Trossaert, O. Zekavat, E. Zetterberg, A. Federici   +34 more
semanticscholar   +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