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Protein C or Protein S deficiency associates with paradoxically impaired platelet-dependent thrombus and fibrin formation under flow.
Res Pract Thromb Haemost
February 2022
Background: Low plasma levels of protein C or protein S are associated with venous thromboembolism rather than myocardial infarction. The high coagulant activity in patients with thrombophilia with a (familial) defect in protein C or S is explained by defective protein C activation, involving thrombomodulin and protein S. This causes increased plasmatic thrombin generation.
View Article and Find Full Text PDFThrombomodulin, an Unexpected New Player in Endothelial Cell Invasion During Angiogenesis.
Arterioscler Thromb Vasc Biol
May 2021
Centre de Recherche Cardio-Thoracique de Bordeaux, Université de Bordeaux, INSERM, U1045, F-33000 Bordeaux. France.
Thrombin cleaves and activates the protease-activated receptor 2 dependent on thrombomodulin co-receptor availability.
Thromb Res
May 2019
Institute of Intensive Care Medicine, University Hospital Zurich, University of Zurich, Zurich, Switzerland. Electronic address:
Introduction: Protease-activated receptors (PARs) evolved to react to extracellular proteolytic activity. In mammals, three of the four PARs (PAR1, PAR3, and PAR4) that are expressed respond to the prototypical procoagulant enzyme thrombin, whereas PAR2 was assumed to resist activation by thrombin. To date, involvement of cell surface thrombin-recruiting co-receptors such as thrombomodulin (TM), which potentially facilitates PAR2 cleavage, has not been addressed.
View Article and Find Full Text PDFThrombin generation and other coagulation parameters in a patient with homozygous congenital protein S deficiency on treatment with rivaroxaban.
Int J Hematol
February 2016
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Università degli Studi di Milano, Via Pace 9, 20122, Milan, Italy.
Rivaroxaban, which targets factor Xa and does not reduce proteins C/S, was chosen to treat a 6-year-old girl with homozygous protein S (PS) deficiency who developed skin necrosis while on warfarin. Owing to the lack of experience with rivaroxaban in children, the girl was started with 5 mg once-daily, which was gradually increased to 40 mg daily. The increasing dosage was driven by the need to avoid recurrence of skin necrosis.
View Article and Find Full Text PDFThromboembolic complications in Fontan patients: population-based prevalence and exploration of the etiology.
Pediatr Cardiol
February 2013
Department of Cardiology, Rigshospitalet, Blegdamsvej 9, Section 2014, 2100 Copenhagen, Denmark.
After the Fontan procedure, patients face an increased risk for thromboembolic events (TE). The etiology for this increased thrombogenecity is incompletely understood. This study aimed to determine the prevalence of TE in Danish Fontan patients and to bring new insights into the etiology of TE.
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