737 results match your criteria adamts13 levels


A mild deficiency of ADAMTS13 is associated with severity in COVID-19: comparison of the coagulation profile in critically and noncritically ill patients.

Blood Coagul Fibrinolysis 2021 Jul 23. Epub 2021 Jul 23.

Department of Hematology Department of Anesthesiology and Reanimation Intensive Care Unit, University General Hospital Gregorio Marañon Universidad Complutense de Madrid Instituto de Investigación Sanitaria Gregorio Marañon, Madrid, Spain.

Early descriptions of COVID-19 associated coagulopathy identified it as a disseminated intravascular coagulation (DIC). However, recent studies have highlighted the potential role of endothelial cell injury in its pathogenesis, and other possible underlying mechanisms are being explored. This study aimed to analyse the coagulation parameters of critically and noncritically ill patients with COVID-19 bilateral pneumonia, determine if coagulation factors consumption occurs and explore other potential mechanisms of COVID-19 coagulopathy. Read More

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Challenges in management of atypical hemolytic uremic syndrome: Bottle neck in resource limited settings.

Transfus Clin Biol 2021 Jul 17. Epub 2021 Jul 17.

Department of Transfusion Medicine, Government Medical College and Hospital, Chandigarh, India. Electronic address:

Treatment of atypical hemolytic uremic syndrome cases is challenging right from establishing correct and timely diagnosis to execution of management protocol. A seven-year-old male child from poor socioeconomic status was admitted with chief complaints of fever, 3 episodes of vomiting and passage of cola coloured urine. Based on clinical and laboratory findings, diagnosis was established. Read More

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Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.

Med Clin (Barc) 2021 Jul 12. Epub 2021 Jul 12.

Servicio de Transfusión. Servicio de Hematología. Complexo Hospitalario Universitario de Ourense, Galicia, España. Electronic address:

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. Read More

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Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID-19 patients.

J Thromb Haemost 2021 Jul 5. Epub 2021 Jul 5.

Service d'Hématologie biologique, Hôpital Lariboisière, AP-HP.Nord, Université de Paris, Paris, France.

Background: Critically ill patients with coronavirus disease 2019 (COVID-19) are prone to developing macrothrombosis and microthrombosis. COVID-19 has been reported to be rarely associated with thrombotic microangiopathies. A disintegrin and metalloprotease with thrombospondin type I repeats, member 13 (ADAMTS13) severe deficiency, the hallmark of thrombotic thrombocytopenic purpura (TTP), induces the formation of platelet, unusually large von Willebrand factor (VWF) multimer microthrombi. Read More

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Effect of red blood cell transfusion on inflammation, endothelial cell activation and coagulation in the critically ill.

Vox Sang 2021 Jul 1. Epub 2021 Jul 1.

Department of Intensive Care Medicine and Laboratory of Experimental Intensive Care and Anesthesiology, Amsterdam UMC, Location VUmc, University of Amsterdam, Amsterdam, The Netherlands.

Background And Objectives: Red blood cell (RBC) transfusion is a frequently applied intervention in an intensive care unit. However, transfusion is associated with adverse outcomes including organ failure and thrombo-embolic events. Mechanisms of these effects are not known but may be related to activation of the endothelium or of the coagulation or inflammatory system. Read More

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Pro-coagulant imbalance in patients with community acquired pneumonia assessed on admission and one month after hospital discharge.

Clin Chem Lab Med 2021 Jul 1. Epub 2021 Jul 1.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan and Fondazione Luigi Villa, Milan, Italy.

Objectives: Patients hospitalized because of community-acquired-pneumonia (CAP) are at risk of cardiovascular diseases. Although plasma procoagulant imbalance play a role, mechanisms are not completely understood. We aimed to investigate whether there is a measurable state of procoagulant imbalance following inflammation determined by CAP. Read More

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[Correlation of serum ADAMTS13 and TSP1 levels with myocardial injury and prognosis in patients with acute coronary syndrome].

Nan Fang Yi Ke Da Xue Xue Bao 2021 May;41(5):710-715

Department of Cardiovascular Disease, First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, China.

Objective: To investigate serum levels of von Willebrand factor lytic protease (ADAMTS13) and thrombospondin-1 (TSP1) in patients with different types of acute coronary syndrome (ACS) and their correlation with the patients' clinical prognosis.

Objective: According to their disease history, results of angiography and clinical biochemical tests, a total of 405 patients undergoing coronary angiography, were divided into unstable angina (UAP) group (=215), acute myocardial infarction (AMI) group (=96), and angiographically normal group (=94). Serum ADAMTS13 and TSP1 levels were detected in all the patients, who were followed up for 15 months to evaluate the occurrence of long-term major cardiac adverse events (MACE). Read More

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Von Willebrand factor, ADAMTS13 and mortality in dialysis patients.

BMC Nephrol 2021 Jun 16;22(1):222. Epub 2021 Jun 16.

Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands.

Background: Von Willebrand Factor (VWF) multimers are cleaved into smaller and less coagulant forms by the metalloprotease ADAMTS13. The aim of this study was to investigate the association between VWF and ADAMTS13 and mortality in dialysis patients.

Methods: We prospectively followed 956 dialysis patients. Read More

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ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.

J Thromb Haemost 2021 Aug 20;19(8):1914-1921. Epub 2021 Jun 20.

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland.

Background: Consistent with fulminant endothelial cell activation, elevated plasma von Willebrand factor (VWF) antigen levels have been reported in patients with COVID-19. The multimeric size and function of VWF are normally regulated through A Disintegrin And Metalloprotease with ThrombSpondin Motif type 1 motif, member 13 (ADAMTS-13)--mediated proteolysis.

Objectives: This study investigated the hypothesis that ADAMTS-13 regulation of VWF multimer distribution may be impaired in severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection contributing to the observed microvascular thrombosis. Read More

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Current prophylactic plasma infusion protocols do not adequately prevent long-term cumulative organ damage in the Japanese congenital thrombotic thrombocytopenic purpura cohort.

Br J Haematol 2021 Jul 28;194(2):444-452. Epub 2021 May 28.

Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan.

Congenital thrombotic thrombocytopenic purpura (cTTP), known as Upshaw-Schulman syndrome, is an ultrarare thrombotic disorder caused by ADAMTS13 gene mutations; however, its long-term outcomes have not been widely studied. A questionnaire survey was administered to physicians of patients in the Japanese cTTP registry to characterise these outcomes. We analysed 55 patients in remission, with 41 cases receiving prophylactic fresh frozen plasma (FFP; median dosage: 13·2 ml/kg per month) and 14 receiving on-demand FFP. Read More

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Early ADAMTS13 testing associates with pre-eclampsia occurrence in antiphospholipid syndrome.

Thromb Res 2021 07 27;203:101-109. Epub 2021 Apr 27.

Department of Gynaecology and Obstetrics, First Moscow State Medical University (Sechenov University), Russian Federation; Department of Haematology, CHU Nîmes, Univ Montpellier, Nîmes, France; Faculty of Pharmaceutical and Biological Sciences, Montpellier University, Montpellier, France; UA 011 INSERM- Université de Montpellier, Institut Desbrest d'Epidémiologie et de Santé Publique, Montpellier, France. Electronic address:

Introduction: Women with obstetric antiphospholipid syndrome (oAPS) still develop placental diseases, mainly pre-eclampsia (PEcl), which diagnosis is associated with reduced ADAMTS13 levels. Testing ADAMTS13 in newly pregnant oAPS may provide evidence for risk stratification.

Materials And Methods: We retrospectively investigated the prognostic value of ADAMTS13 activity, antigen and antibodies on stored plasma samples obtained prior to beginning low-molecular weight heparin-low dose aspirin treatment in 513 oAPS women. Read More

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Plasma Levels of Big Endothelin-1 Are Associated with Renal Insufficiency and In-Hospital Mortality of Immune Thrombotic Thrombocytopenic Purpura.

Thromb Haemost 2021 May 13. Epub 2021 May 13.

Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, Kansas, United States.

Immune thrombotic thrombocytopenic purpura (iTTP) is caused by severe deficiency of plasma ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity. Despite advances in early diagnosis and management, the mortality rate of acute iTTP remains high in a large part of world where access to some of the most novel therapies is limited. To determine the role of plasma big endothelin-1 (bigET-1) or its bioactive product ET-1 as a biomarker and/or a pathogenic factor in acute iTTP, plasma levels of bigET-1 were determined using an immunoassay in patients with iTTP on admission and during remission, as well as in healthy controls; moreover, the biological effect of ET-1 in thrombus formation was determined by a microfluidic assay. Read More

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Complement levels at admission as a reflection of coronavirus disease 2019 (COVID-19) severity state.

J Med Virol 2021 09 19;93(9):5515-5522. Epub 2021 May 19.

Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Complement system hyperactivation has been proposed as a potential driver of adverse outcomes in severe acute respiratory syndrome coronavirus 2 infected patients, given prior research of complement deposits found in tissue and blood samples, as well as evidence of clinical improvement with anticomplement therapy. Its role in augmenting thrombotic microangiopathy mediated organ damage has also been implicated in coronavirus disease 2019 (COVID-19). This study aimed to examine associations between complement parameters and progression to severe COVID-19 illness, as well as correlations with other systems. Read More

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September 2021

Von Willebrand factor collagen-binding capacity predicts in-hospital mortality in COVID-19 patients: insight from VWF/ADAMTS13 ratio imbalance.

Angiogenesis 2021 08 11;24(3):407-411. Epub 2021 May 11.

Université de Paris, Innovative Therapies in Haemostasis, INSERM, 75006, Paris, France.

Background: Microthrombosis is a hallmark of COVID-19. We previously described von willebrand factor (VWF) and their high molecular weight multimers (HMWMs) as potential trigger of microthrombosis.

Objectives: Investigate VWF activity with collagen-binding assay and ADAMTS13 in COVID-19. Read More

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The association of von willebrand factor and its cleaving protease (ADAMTS13) with health behaviours in young black and white adults: the African-PREDICT study.

Biomarkers 2021 May 26:1-9. Epub 2021 May 26.

Hypertension in Africa Research Team (HART), North-West University, Potchefstroom, South Africa.

To reduce cardiovascular risks imposed by Von Willebrand factor (vWF) and ADAMTS13 from young ages, knowledge on health behaviours that may affect their concentrations is essential. We therefore determined whether circulating vWF antigen and ADAMTS13 associate with health behaviours. We included 1196 black and white healthy adults aged 20-30 years and used questionnaires for socio-economic, tobacco and alcohol use data. Read More

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Serum and cerebrospinal fluid host proteins indicate stroke in children with tuberculous meningitis.

PLoS One 2021 30;16(4):e0250944. Epub 2021 Apr 30.

Department of Paediatrics and Child Health, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa.

Introduction: Stroke is a common complication in children with tuberculous meningitis (TBM). Host proteins may give us insight into the mechanisms of stroke in TBM and serve as biomarkers for detection of stroke, however, they have not been widely explored. In this study, we compared the concentrations of cerebrospinal fluid (CSF) and serum proteins between children who had TBM-related stroke and children with TBM without stroke. Read More

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Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.

Semin Thromb Hemost 2021 Jun 23;47(4):400-418. Epub 2021 Apr 23.

Section of Clinical Biochemistry, University of Verona, Verona, Italy.

von Willebrand factor (VWF) is a large adhesive multimeric protein involved in hemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect of VWF can lead to von Willebrand disease (VWD) and cause bleeding. Read More

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Von Willebrand factor and ADAMTS13 activity as clinical severity markers in patients with COVID-19.

Authors:
A Marco P Marco

J Thromb Thrombolysis 2021 Apr 17. Epub 2021 Apr 17.

Thrombosis and Hemostasis Department, Hematology Service, University General Hospital in Alicante, Alicante, Spain.

The coronavirus disease 2019 (COVID-19) increases thrombotic risk. The mechanisms that lead to this prothrombotic state are not well established. The main aim was to evaluate the von Willebrand factor (VWF) antigen and plasma ADAMTS13 activity as endothelial injury markers in COVID-19. Read More

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Post-cardiac surgery thrombotic thrombocytopenic purpura: presence of anti-ADAMTS13 autoantibodies at preoperative baseline.

Platelets 2021 Apr 14:1-5. Epub 2021 Apr 14.

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Thrombotic thrombocytopenic purpura (TTP) rarely complicates acute inflammatory conditions such as surgery, including post-cardiac surgery. Review of 32 previously-reported cases of post-cardiac surgery TTP indicates that this disorder often occurs as early as 2-3 days following surgery, which seems too soon to implicate new formation of anti-ADAMTS13 autoantibodies as a consequence of surgery itself. We diagnosed post-cardiac surgery TTP in a 60-year-old female that began approximately 3 days post-coronary artery bypass surgery in which anti-ADAMTS13 autoantibodies were implicated. Read More

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Prognostic Biomarkers for Thrombotic Microangiopathy after Acute Graft-versus-Host Disease: A Nested Case-Control Study.

Transplant Cell Ther 2021 04 16;27(4):308.e1-308.e8. Epub 2020 Dec 16.

Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington; Division of Nephrology, Seattle Children's Hospital, Seattle, Washington; Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington.

Transplantation-associated thrombotic microangiopathy (TA-TMA) is a complication of allogeneic hematopoietic cell transplantation (HCT) that often occurs following the development of acute graft-versus-host disease (aGVHD). In this study, we aimed to identify early TMA biomarkers among patients with aGVHD. We performed a nested-case-control study from a prospective cohort of allogeneic HCT recipients, matching on the timing and severity of antecedent aGVHD. Read More

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Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients.

J Pediatr 2021 Aug 7;235:196-202. Epub 2021 Apr 7.

IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy; Sechenov First Moscow State Medical University, Moscow, Russian Federation.

Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).

Study Design: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS. Read More

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Targeting von Willebrand factor in liver diseases: A novel therapeutic strategy?

J Thromb Haemost 2021 06 3;19(6):1390-1408. Epub 2021 May 3.

Department of Pathobiology & Diagnostic Investigation, Michigan State University, East Lansing, MI, USA.

Acute and chronic liver disease are associated with substantial alterations in the hemostatic system. Evidence from both experimental and clinical studies suggests that anticoagulants slow the progression of liver disease. Efficacy of those anticoagulant drugs is, in part, attributed to a reduction of microthrombi formation within the liver. Read More

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Low ADAMTS13 Activity Correlates with Increased Mortality in COVID-19 Patients.

TH Open 2021 Jan 9;5(1):e89-e103. Epub 2021 Mar 9.

Department of Pathology Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, United States.

The causes of coagulopathy associated with coronavirus disease 2019 (COVID-19) are poorly understood. We aimed to investigate the relationship between von Willebrand factor (VWF) biomarkers, intravascular hemolysis, coagulation, and organ damage in COVID-19 patients and study their association with disease severity and mortality. We conducted a retrospective study of 181 hospitalized COVID-19 patients randomly selected with balanced distribution of survivors and nonsurvivors. Read More

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January 2021

Recombinant ADAMTS13 reduces abnormally up-regulated von Willebrand factor in plasma from patients with severe COVID-19.

Thromb Res 2021 05 18;201:100-112. Epub 2021 Feb 18.

School of Cellular and Molecular Medicine, University of Bristol, Bristol, United Kingdom; University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom. Electronic address:

Thrombosis affecting the pulmonary and systemic vasculature is common during severe COVID-19 and causes adverse outcomes. Although thrombosis likely results from inflammatory activation of vascular cells, the mediators of thrombosis remain unconfirmed. In a cross-sectional cohort of 36 severe COVID-19 patients, we show that markedly increased plasma von Willebrand factor (VWF) levels were accompanied by a partial reduction in the VWF regulatory protease ADAMTS13. Read More

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Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.

PLoS One 2021 19;16(2):e0247017. Epub 2021 Feb 19.

Department of Health Sciences, Thrombosis and Atherosclerosis Research Institute (TaARI), McMaster University, Hamilton, Ontario, Canada.

Sepsis is a life-threatening disease characterized by excessive host response to infection that can lead to activation of the coagulation system. Von Willebrand Factor (VWF) and ADAMTS13 are important regulators of hemostasis and their dysregulation during sepsis progression is not well understood. Herein we characterize ADAMTS13 and VWF in septic and non-septic patients. Read More

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February 2021

Identification of Biomarkers in Patients with Thrombotic Thrombocytopenic Purpura Presenting with Large and Small Ischemic Stroke.

Cerebrovasc Dis Extra 2021 18;11(1):29-36. Epub 2021 Feb 18.

Department of Pathology, The University of Alabama at Birmingham, Birmingham, Alabama, USA.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder resulting in organ damage including ischemic strokes. We sought to characterize the neuroimaging patterns of stroke in a large cohort of patients with immune-mediated TTP (iTTP) and determined their associations with clinical and laboratory parameters and outcomes.

Methods: We analyzed the Alabama TTP Registry who had laboratory confirmation of acute iTTP. Read More

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von Willebrand Factor Multimer Formation Contributes to Immunothrombosis in Coronavirus Disease 2019.

Crit Care Med 2021 05;49(5):e512-e520

Medical Department I, University Hospital Marien Hospital Herne, Ruhr-University Bochum, Herne, Germany.

Objectives: Prevention and therapy of immunothrombosis remain crucial challenges in the management of coronavirus disease 2019, since the underlying mechanisms are incompletely understood. We hypothesized that endothelial damage may lead to substantially increased concentrations of von Willebrand factor with subsequent relative deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13).

Design: Prospective controlled cross-over trial. Read More

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No Evidence for Classic Thrombotic Microangiopathy in COVID-19.

J Clin Med 2021 Feb 9;10(4). Epub 2021 Feb 9.

Institute of Clinical Chemistry and Laboratory medicine, University Medical Center of the Johannes Gutenberg University Mainz, 55131 Mainz, Germany.

Background: Coronavirus disease-2019 (COVID-19) triggers systemic infection with involvement of the respiratory tract. There are some patients developing haemostatic abnormalities during their infection with a considerably increased risk of death.

Materials And Methods: Patients ( = 85) with SARS-CoV-2 infection attending the University Medical Center, Mainz, from 3 March to 15 May 2020 were retrospectively included in this study. Read More

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February 2021