Results 141 to 150 of about 56,101 (281)

Schematic domain structure of full-length VWF and recombinant VWF fragments.

, 2013
The domain structure of human preproVWF is shown above the structures of recombinant VWF fragments designed in this study. ADAMTS-13 cleaves the Y1605-M1606 peptidyl bond in the A2 domain (D1459-L1668).
Ningzheng Dong (328283), Changgeng Ruan (328282), Jingyu Zhang (256203), Yiming Zhao (348349), Zhenni Ma (328280), Jian Su (86223), Anyou Wang (281729), Fang Liu (13271), Fei Shen (348351)   +8 more
core   +1 more source

Contrasting Approaches in the Implementation of GRADE Methodology in Guidelines for Haemophilia and Von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The 2024 ISTH clinical practice guideline (CPG) for treatment of congenital haemophilia, the NBDF‐McMaster Guideline on Care Models for Haemophilia Management, and ASH ISTH NBDF WFH guidelines on the diagnosis and management of VWD all utilised GRADE methodology.
Mark W. Skinner, Manuela Albisetti, Jesús Ardila, Jan Astermark, Jan Blatny, Manuel Carcao, Pratima Chowdary, Nathan T. Connell, Miguel Crato, Yesim Dargaud, Roseline d'Oiron, Amy L. Dunn, Miguel A. Escobar, Carmen Escuriola‐Ettingshausen, Emna Gouider, Annie Harroche, Cedric Hermans, Victor Jimenez‐Yuste, Radoslaw Kaczmarek, Gili Kenet, Liane Khoo, Robert Klamroth, Florian Langer, David Lillicrap, Johnny Mahlangu, Christoph Male, Tadashi Matsushita, Sandrine Meunier, Wolfgang Miesbach, Beatrice Nolan, Johannes Oldenburg, Brian O'Mahony, Margareth Ozelo, Glenn F. Pierce, Gloria Ramos, Michael Recht, Olivia Romero‐Lux, Dawn Rotellini, Rita C. Santoro, Tammuella C. Singleton, Alok Srivastava, Sophie Susen, Kate Talks, Huyen Tran, Leonard A. Valentino, Jerzy Windyga, Renchi Yang, Maria Elisa Mancuso, also on behalf of AICE, AHAD‐AP, AHCDC, ATHN, CLAHT, EHC, French CRH & MHEMO, HTCCNC, NBDF, PEDNET, UKHCDO, WFH   +59 more
wiley   +1 more source

GrM disrupts FVIII binding to VWF.

, 2013
(A) Purified plasma VWF (1 µg/mL) VWF was incubated with indicated concentration of GrM, GrB, GrM-SA or GrB-SA in Tris (pH 7.4) for 1 hour at 37°C and subsequent FVIII binding was assessed in an ELISA setup.
Martine J. Hollestelle (345019), Philip G. de Groot (167182), Tom Sprong (278692), Peter J. Lenting (133252), Niels Bovenschen (345021), Ka Wai Lai (345020), Marcel van Deuren (15650)   +6 more
core   +1 more source

Performing Large‐Scale Genetic Analysis in the Bleeding Disorders Community

Haemophilia, EarlyView.
ABSTRACT Inherited bleeding disorders encompass a diverse group of conditions caused by genetic defects affecting coagulation factors, fibrinogen, von Willebrand factor, or platelet function. Despite major advances in quantitative and functional laboratory assays, a substantial diagnostic gap remains, particularly in patients with mild or atypical ...
Anna R. Blankstein, Sterre P. E. Willems, Saskia E. M. Schols, Rosanna Asselta, Gillian Lowe, Neil V. Morgan, Paula D. James   +6 more
wiley   +1 more source

vWF sequences 2

, 2016
Nexus file containing phased alleles from vWF sequences of Sunda shelf Crocidura.
Handika, Heru, Demos, Terrence C., Thomas C. Giarla (3199704), Maharadatunkamsi (3199695), Heru Handika (3199701), Kevin C. Rowe (223136), Terrence C. Demos (798528), Jacob A. Esselstyn (214666), Rowe, Kevin C., Anang S. Achmadi (3199698), Giarla, Thomas C., Esselstyn, Jacob A., Maharadatunkamsi, Achmadi, Anang S.   +13 more
core   +1 more source

Rare Bleeding Disorders and Bleeding Disorder of Unknown Cause: Current Understanding and Recent Developments

Haemophilia, EarlyView.
ABSTRACT Rare bleeding disorders (RBDs) represent a diverse group of inherited conditions involving coagulation factors or platelets. These conditions, such as Glanzmann thrombasthenia (GT) or severe coagulation factor deficiencies, are uncommon. In contrast, bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion without an identifiable ...
Alessandro Casini, Robert Klamroth, Bauke Haisma, Dino Mehic, Saskia E. M. Schols   +4 more
wiley   +1 more source

The Swiss Haemophilia Registry–Report From the First 8 Years

Haemophilia, EarlyView.
ABSTRACT Introduction Patient registries capture disease related information and provide a valuable source for real‐world data on rare diseases and their management. The Swiss Haemophilia Registry (SHR) was established in 2015 on the basis of a new Swiss federal human research act.
Alessandra Bosch, Lorenzo Alberio, Pierre Fontana, Lukas Graf, Johanna A. Kremer Hovinga, Nicolas von der Weid, Mattia Rizzi, Manuela Albisetti, the Swiss Haemophilia Network   +8 more
wiley   +1 more source

Monitoring Extended Half‐Life Factor VIII and IX Concentrates: Performance in Routine Clotting‐and Thrombin Generation Assays

Haemophilia, EarlyView.
ABSTRACT Introduction Monitoring extended half‐life (EHL) factor (F) VIII and FIX products is complex, as discrepant results are obtained in different laboratory assays. Aim To provide an overview on assay accuracy based on a re‐assessment of previously published data as well as laboratory results of EHL spiking experiments for specific factor assays ...
An K. Stroobants, Eva‐Leonne Göttgens, Yvonne M. C. Henskens, Joop H. Jansen, Saskia E. M. Schols, Sanna R. Rijpma   +5 more
wiley   +1 more source

Management of COVID-19 associated coagulopathy in critically ill patients and the risk of acquired von willebrand syndrome

Scientific Reports
This study investigated the interplay between thrombosis and hemorrhage in critically ill COVID-19 patients, particularly those on extracorporeal membrane oxygenation (ECMO).
Hiroyasu Ishikura, Yuhei Irie, Maiko Nakashio, Junichi Maruyama, Yoshihiko Nakamura, Takahiro Yamagaito, Maho Yoshida, Hiroki Hatomoto, Shintaro Yamasaki, Kota Hoshino   +9 more
doaj   +1 more source

Stul polymorphisms in the vWF gene [PDF]

Nucleic Acids Research, 1990
A, Inbal, R I, Handin
openaire   +2 more sources