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View article: The effects of balanced crystalloid versus plasma on endothelial injury, systemic inflammation, and coagulation in experimental endotoxaemia: a randomised human volunteer study
The effects of balanced crystalloid versus plasma on endothelial injury, systemic inflammation, and coagulation in experimental endotoxaemia: a randomised human volunteer study Open
In human endotoxaemia, plasma resuscitation reduced leucocyte and neutrophil levels but did not reduce glycocalyx degradation, when compared with equal volume resuscitation with a balanced crystalloid solution.
View article: A novel p.C1130S mutation in a Finnish family with a complex phenotype of von Willebrand disease
A novel p.C1130S mutation in a Finnish family with a complex phenotype of von Willebrand disease Open
Not available.
View article: Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS
Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS Open
Allosteric regulation of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type-1 motif, member 13) activity involves an interaction between its spacer (S) and 2 complement C1r/C1s, Uegf and BMP1 (CUB; CUB1-2) domains to ke…
View article: Limited Impact of Drone Transport of Blood on Platelet Activation
Limited Impact of Drone Transport of Blood on Platelet Activation Open
The rapid transportation of blood samples and blood products using drones has high potential in the medical sector. However, before this can be implemented, sufficient evidence that drone transportation is not affecting clinical outcomes i…
View article: OC 06.4 Therapeutic Efficacy of aNovel Humanized anti-ADAMTS13 Antibody to Treat Left Ventricular Assist Device-Induced Acquired Von Willebrand Syndrome
OC 06.4 Therapeutic Efficacy of aNovel Humanized anti-ADAMTS13 Antibody to Treat Left Ventricular Assist Device-Induced Acquired Von Willebrand Syndrome Open
Results: Eighteen studies were included in the final review for a total of 45 patients.The median age was 78 years (range: 21-93).The vast majority (75.6%) were males.Presenting bleeding symptoms were widely variable; the most common were …
View article: Antithrombin p.Thr147Ala: The First Founder Mutation in People of African Origin Responsible for Inherited Antithrombin Deficiency
Antithrombin p.Thr147Ala: The First Founder Mutation in People of African Origin Responsible for Inherited Antithrombin Deficiency Open
Background Hereditary antithrombin deficiency is a rare autosomal-dominant disorder predisposing to recurrent venous thromboembolism (VTE). To date, only two founder mutations have been described. Objectives We investigated the antithrombi…
View article: Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura
Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura Open
Recently, we showed that during the acute phase of immune-mediated thrombotic thrombocytopenic purpura (iTTP), ADAMTS13 circulates in an open conformation. Although the cause of this conformational change in acute iTTP remains elusive, ADA…
View article: Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function
Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function Open
Plasma ADAMTS13 circulates in a folded conformation that is stabilized by an interaction between the central Spacer domain and the C-terminal CUB (complement components C1r and C1s, sea urchin protein Uegf, and bone morphogenetic protein-1…
View article: Generation of anti-idiotypic antibodies to detect anti-spacer antibody idiotopes in acute thrombotic thrombocytopenic purpura patients
Generation of anti-idiotypic antibodies to detect anti-spacer antibody idiotopes in acute thrombotic thrombocytopenic purpura patients Open
In autoantibody-mediated autoimmune diseases, autoantibody profiling allows patients to be stratified and links autoantibodies with disease severity and outcome. However, in immune-mediated thrombotic thrombocytopenic purpura (iTTP) patien…
View article: Differences in von Willebrand factor function in type 2A von Willebrand disease and left ventricular assist device‐induced acquired von Willebrand syndrome
Differences in von Willebrand factor function in type 2A von Willebrand disease and left ventricular assist device‐induced acquired von Willebrand syndrome Open
VWF:CB/VWF:Ag or VWF:RCo/VWF:Ag analysis allows detection of impaired VWF function in VWD type 2A but not always in LVAD-induced aVWS patients. In contrast, VWF multimeric analysis allows detection of the loss of HMW VWF multimers in both …
View article: Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura
Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura Open
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency, the presence of anti-ADAMTS13 autoantibodies and a…
View article: Issue Information
Issue Information Open
Impact of white blood cells on thrombotic risk in patients with optimized platelet count in essential thrombocythemia Efficacy and safety of rivaroxaban compared to enoxaparin in treatment of cancer-associated venous thromboembolism Transi…
View article: Child‐onset thrombotic thrombocytopenic purpura caused by p.R498C and p.G259PfsX133 mutations in ADAMTS13
Child‐onset thrombotic thrombocytopenic purpura caused by p.R498C and p.G259PfsX133 mutations in ADAMTS13 Open
Introduction Patients suffering from congenital thrombotic thrombocytopenic purpura (cTTP) have a deficiency in ADAMTS13 due to mutations in their ADAMTS13 gene. Objective The aim of this study was to determine ADAMTS13 parameters (activit…
View article: Anti-ADAMTS13 Antibodies and a Novel Heterozygous p.R1177Q Mutation in a Case of Pregnancy-Onset Immune-Mediated Thrombotic Thrombocytopenic Purpura
Anti-ADAMTS13 Antibodies and a Novel Heterozygous p.R1177Q Mutation in a Case of Pregnancy-Onset Immune-Mediated Thrombotic Thrombocytopenic Purpura Open
In this study, we investigated a case of pregnancy-onset thrombotic thrombocytopenic purpura (TTP). The patient had severely decreased ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13) activity levels…
View article: Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease
Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease Open
Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is kn…
View article: N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura
N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura Open
Key Points Prophylactic administration of NAC was effective in preventing severe TTP signs in mice by reducing the VWF multimer size. In both mice and baboons, NAC was not effective in resolving preexisting TTP signs, as thrombus resolutio…
View article: Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura
Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura Open
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate devel…
View article: Platelet-derived VWF is not essential for normal thrombosis and hemostasis but fosters ischemic stroke injury in mice
Platelet-derived VWF is not essential for normal thrombosis and hemostasis but fosters ischemic stroke injury in mice Open
Key Points Platelet-derived VWF alone mediates full ischemic stroke injury in mice via a GPIb-dependent mechanism. Platelet-derived VWF does not significantly contribute to normal thrombosis and hemostasis in mice.