Results 151 to 160 of about 147,268 (335)

Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura.

New England Journal of Medicine, 1998
H. Tsai, E. Lian
semanticscholar   +1 more source

Proteolysis at the extracellular matrix interface: Molecular architects and regulators in health and disease

The FEBS Journal, EarlyView.
The extracellular matrix (ECM) is a dynamic scaffold that orchestrates tissue architecture and cellular communication. A critical but underexplored interplay between proteases and cluster of differentiation molecules (CD) governs ECM turnover and directs cell fate.
David Jurnečka, Matija Hrovatin, Bor Bokalj, Boris Turk   +3 more
wiley   +1 more source

von Willebrand factor propeptide: biology and clinical utility.

Blood, 2015
S. Haberichter
semanticscholar   +1 more source

Proteolytic remodelling of the extracellular matrix by pericytes

The FEBS Journal, EarlyView.
Pericytes are specialised perivascular cells intimately connected with endothelial cells and essential for the maintenance of vascular beds. They contribute to the formation and remodelling of the extracellular matrix by actively secreting proteases and protease inhibitors.
Tina Burkhard, Ella Milne, Kate Qian, Paola Campagnolo, Salvatore Santamaria   +4 more
wiley   +1 more source

Evaluation of the Clinical Status of Patients with Inherited Bleeding Disorders in Diyala-Iraq

Diyala Journal of Medicine, 2019
Background: Inherited bleeding disorders are rare diseases that are both complicated and expensive to manage, they are classified into: coagulation factor disorders, vessel wall defect and platelet disorders.
Imad Ahmed Lateef
doaj   +2 more sources

Oral post-surgical complications in patients with hemophilia and von Willebrand disease

Hematology, Transfusion and Cell Therapy
Objective: To determine the prevalence of post-surgical complications in patients with hemophilia and von Willebrand disease. Methods: A prospective, cross-sectional study with descriptive and exploratory data analysis was conducted at the outpatient ...
Luisa Catarina Porfirio de Sousa, Elanne Cristina Garcia da Costa Félix, Wagner Hespanhol, Raquel dos Santos Pinheiro   +3 more
doaj   +1 more source

Chemical Chaperones Improve Protein Secretion and Rescue Mutant Factor VIII in Mice with Hemophilia A.

, 2012
nefficient intracellular protein trafficking is a critical issue in the pathogenesis of a variety of diseases and in recombinant protein production.
Tonn, Torsten, Milanov, Peter, Schüttrumpf, Jörg, Seifried, Erhard, Patricia Quade-Lyssy, Quade-Lyssy, Patricia, Jörg Schüttrumpf, Roth, Stefanie D., Simpson, Jeremy C., Christopher Ungerer, Erhard Seifried, Abriss, Daniela, Pepperkok, Rainer, Rainer Pepperkok, Stefanie D Roth, Jeremy C Simpson, Torsten Tonn, Ungerer, Christopher, Peter Milanov, Daniela Abriss   +19 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

Doença de Von Willebrand: conhecimentos necessários ao profissional quanto ao tratamento cirúrgico em odontologia

, 2018
Objetivo: realizar uma revisão de literatura acerca da conduta terapêutica do cirurgião-dentista frente a um paciente portador da doença de von Willebrand que necessite de procedimento cirúrgico.
Gomes, Erick Agostinho Cucco
core   +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