Results 111 to 120 of about 52,334 (267)

Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease

Clinics, 2013
OBJECTIVE: To analyze the preoperative plasma antigenic concentration and activity of von Willebrand factor and its main cleaving protease ADAMTS-13 in pediatric patients with cyanotic congenital heart disease undergoing surgical treatment and ...
Rosangela P.S. Soares, Sérgio P. Bydlowski, Marcelo B. Jatene, Janete Ferreira Hironaka, Antonio Augusto Lopes   +4 more
doaj  

Von Willebrand disease: A century of progress

Research and Practice in Thrombosis and Haemostasis
One hundred years after the initial description of von Willebrand disease, originally referred to as pseudohemophilia, this article is a tribute to Dr Erik von Willebrand and a testament to the progress in our understanding of von Willebrand factor. Main
Caterina Casari, Brooke Sadler, Sophie Susen, Riitta Lassila   +3 more
doaj   +1 more source

Defining the role of αC helix interactions in the activation of the integrin αI domain

The FEBS Journal, EarlyView.
The α2I domain of the α2β1 integrin contains a distinctive αC helix and a conserved Arg288‐Glu318 ion pair that stabilizes the closed state of the domain. Disruption of this ion pair in the activated α2I variant unwinds the αC helix, which allows interaction with additional collagen‐binding sites increasing collagen binding.
Liisa Pösö, Tomi T. Airenne, Jarmo Käpylä, Hongmin Tu, Eva Bligt‐Lindén, Vimal Parkash, Jyrki Heino, Tiina A. Salminen   +7 more
wiley   +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

Enhancing the Evidence for Care in Underserved Bleeding Disorders Communities

Haemophilia, EarlyView.
ABSTRACT Background Major advances in haemophilia care have not translated equitably across all populations. Individuals with rare bleeding disorders (RBDs), people living in low‐ and lower‐middle‐income countries (LMICs) and women and girls with inherited bleeding disorders (WGWBD) continue to face significant diagnostic, therapeutic and research ...
Johnny Mahlangu
wiley   +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

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

Seventh Åland Island Meeting on von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The seventh Åland Island Meeting on von Willebrand Disease (VWD) was held on the Åland archipelago in Finland, from 26 to 28 September 2024. Aim The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD.
Riitta Lassila, Ruben Bierings, Nathan T. Connell, Fernando F. Corrales‐Medina, Augusto B. Federici, Veronica H. Flood, Floor Heubel‐Moenen, Jill M. Johnsen, Geoffrey Kuppens, Davide Matino, Carolyn Millar, Ingmar Schoen, Anish Sharda, Robert F. Sidonio Jr, Alok Srivastava, Timea Szanto, Angela C. Weyand, Erik Berntorp   +17 more
wiley   +1 more source

Optimizing Perioperative Management of Haemophilia B With rFIX‐FP: Pharmacokinetic Validation of the Hemoptidose Tool

Haemophilia, EarlyView.
Abstract Background Surgical management of haemophilia B requires precise factor IX replacement to ensure adequate haemostasis while optimizing factor consumption. Extended half‐life rFIX‐FP simplifies perioperative management but exhibits substantial pharmacokinetic variability.
Xavier Delavenne, Célia Vollard, Nicolas Drillaud, Sabine Castet, Nicolas Luyck, Emilie Hénin, Julien Denis‐Le Seve, Fabienne Volot, Brigitte Tardy, Stéphanie Désage, Benoît Guillet   +10 more
wiley   +1 more source

Responsiveness and Minimal Important Change of the Haemophilia Activities List in Patients With Inherited Bleeding Disorders

Haemophilia, EarlyView.
ABSTRACT Introduction The Haemophilia Activities List (HAL) is a disease‐specific patient‐reported outcome measure (PROM) that is widely used in both healthcare and research in people with inherited bleeding disorders (PWBD), but information on responsiveness and minimal important change (MIC) is lacking.
Johan Blokzijl, Isolde A. R. Kuijlaars, Martijn F. Pisters, Cindy Veenhof, Janjaap van der Net, Roger E. G. Schutgens, Kathelijn Fischer, Merel A. Timmer   +7 more
wiley   +1 more source