Results 81 to 90 of about 16,303 (230)

DDAVP Challenges in 170 Children With von Willebrand Disease: Response Classification Varies According to Criteria Used

Haemophilia, EarlyView.
ABSTRACT Introduction Individuals diagnosed with types 1/1C/2A/2M von Willebrand disease (VWD) typically undergo a desmopressin (DDAVP) challenge to assess therapeutic benefit. This involves measuring von Willebrand factor antigen (VWF:Ag), activity (VWF:Act; VWF:RCo/VWF:GPIbM/VWF:Ab), and coagulant factor VIII (FVIII:C) levels 0 (pre), 1, and 4‐h post‐
Michael Shu, Caroline Malcolmson, Amanda Fregonas, Tristan E. Knight, Margaret L. Rand, Cindy Wakefield, Vanessa Bouskill, Manuel Carcao   +7 more
wiley   +1 more source

Clinical Outcomes of Efanesoctocog Alfa Prophylaxis in Children With Severe Haemophilia A: Interim Data From XTEND‐ed

Haemophilia, EarlyView.
Lynn Malec, Beatrice Nolan, Anthony K C Chan, Manuela Albisetti, Sheng‐Chieh Chou, Bulent Zulfikar, Mindy Simpson, Liqi Feng, Helena Palmborg, Graham Neill, Lydia Abad‐Franch, Sriya Gunawardena, Karin Fijnvandraat   +12 more
wiley   +1 more source

Underdiagnosis of Von Willebrand Disease: The Silent Majority of Women in Brazil

Haemophilia, EarlyView.
Abstract Introduction Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Although autosomal inheritance predicts a similar distribution between sexes, women are more often diagnosed due to hemostatic challenges associated with menstruation and childbirth.
Yara Maria da Silva Pires, Gabriel Iamassaki Staquicini de Paula, Thyago César da Silva Pires   +2 more
wiley   +1 more source

Deletion of alanine 2201 in the FVIII C2 domain results in mild hemophilia A by impairing FVIII binding to VWF and phospholipids and destroys a major FVIII antigenic determinant involved in inhibitor development

, 2004
The C2 domain of factor VIII (FVIII) mediates FVIII binding to von Willebrand factor (VWF) and phospholipids (PLs), thereby determining the stability and the activity of FVIII.
Benhida, Abdellah, Vermylen, Jozef, Lavend'homme, Renaud, Peerlinck, Kathelijne, Jacquemin, Marc, Saint-Remy, Jean-Marie, Bordet, Jean-Claude, Negrier, Claude, d'Oiron, Roseline, Lavergne, Jean-Maurice   +9 more
core   +1 more source

Regulation of immune responses to therapeutic factor VIII by transplacental delivery of Fc-fused immunodominant factor VIII domains or peptides

Haematologica
Patients with severe hemophilia A (HA) often develop undesired immune responses to therapeutic factor VIII (FVIII) that hamper replacement therapy with FVIII-derived products. The transplacental delivery of two Fc-fused FVIII domains in pregnant HA mice
Alejandra Reyes-Ruiz, Sandrine Delignat, Aurélien Azam, Victoria Daventure, Leslie Dourthe, Angelina Mimoun, Geneviève McCluskey, Maria T. Georgescu, David Lillicrap, Jordan D. Dimitrov, Sebastien Lacroix-Desmazes   +10 more
doaj   +1 more source

Global Diagnostics of Hemophilia and Bleeding Disorders: Insights From World Federation of Hemophilia International External Quality Assessment Scheme Program 2016–2023

Haemophilia, EarlyView.
ABSTRACT Introduction Accurate laboratory diagnosis is essential for the effective management of bleeding disorders. The World Federation of Hemophilia (WFH) International External Quality Assessment Scheme (IEQAS) provides an international platform for comparative evaluation of laboratory performance.
Silmara de Lima Montalvão, Ian Jennings, Dianne P. Kitchen, Christopher Reilly‐Stitt, Glenn F. Pierce, Andreina Tovar, Jayson Martin, Assad Haffar, Steve Kitchen   +8 more
wiley   +1 more source

Fc-fusion technology and recombinant FVIII and FIX in the management of the hemophilias

, 2014
Maria Elisa Mancuso,1 Pier Mannuccio Mannucci21Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, 2Scientific Direction, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, ItalyAbstract: Prophylaxis with regular infusions of ...
Mannucci PM, Mancuso ME
core  

Characterization of Natural Anti-FVIII Antibodies and the Regulatory Role of the Gut Microbiota in FVIII Immunogenicity

, 2023
Patients with hemophilia A bleed spontaneously into the soft tissues and joints due to deficiencies in the coagulation protein Factor VIII (FVIII).
Cormier, Matt
core  

Evaluation of Global Hemostatic Assays in Response to Factor VIII Inhibitors

Clinical and Applied Thrombosis/Hemostasis, 2019
Global hemostatic assays including thromboelastography (TEG), Innovance ETP (endogenous thrombin potential), and Thrombinoscope could measure thrombin generation potential and be useful to guide management of patients with factor VIII (FVIII) inhibitors.
Ping Chen MD, PhD, Jayesh Jani MS, Michael B. Streiff MD, Gang Zheng MD, PhD, Thomas S. Kickler MD   +4 more
doaj   +1 more source

Real‐World Experience With Emicizumab for Haemophilia A From the Physician Perspective in a Real‐World Clinical Setting

Haemophilia, EarlyView.
ABSTRACT Introduction Emicizumab is a bispecific antibody approved for prophylaxis in people with haemophilia A (PwHA). Aim To describe from the physician perspective the characteristics and treatment experiences of PwHA receiving emicizumab. Methods The Adelphi Real World Haemophilia Disease Specific Programme is a cross‐sectional, retrospective data ...
Michael Recht, Chris Blazos, Sophie Lai, Nathan Ball, Ella Morton, Lucy Lee, Juliana M. L. Biondo, Janet S. Lee   +7 more
wiley   +1 more source