11,687 results match your criteria von Willebrand Disease


Presence of vasculature results in faster insulin response in adipocytes in vascularized adipose tissue model.

ALTEX 2019 Mar 11. Epub 2019 Mar 11.

Cell Biology, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

Besides being an energy storage, adipose tissue is an endocrine organ closely associated with vascular system. Human relevant in vitro models are needed to study adipose tissue and related diseases. Vasculature plays a central role in the development and inhibition of adipose tissue related diseases. Read More

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http://dx.doi.org/10.14573/altex.1811271DOI Listing

[Von Willebrand disease in the elderly].

Rev Prat 2018 Dec;68(10):1125-1131

Service d'hématologie biologique, site constitutif du centre de référence maladie rare de la maladie de Willebrand, hôpital Lariboisière, AP-HP, Paris, France.

Von Willebrand disease in the elderly. Von Willebrand disease (VWD) is a rare inherited haemorrhagic disorder, the prevalence of symptomatic individuals is around 1/10 000. Von Willebrand factor level increases with advanced age, explaining a lower frequency and a lower severity of cutaneous haemorrhagic symptoms with aging. Read More

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December 2018
1 Read

Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases.

J Atheroscler Thromb 2019 Mar 12. Epub 2019 Mar 12.

Department of Blood Transfusion Medicine, Nara Medical University.

The blood glycoprotein von Willebrand factor (VWF) plays an important role in hemostasis and thrombosis.VWF is produced and secreted as large multimers by endothelial cells and megakaryocytes. It is then cleaved in a sheer-stress dependent manner by a specific protease, ADAMTS13, into multimers consisting of 2-80 subunits. Read More

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http://dx.doi.org/10.5551/jat.RV17031DOI Listing
March 2019
1 Read

Whole blood ristocetin-induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Background: The haemorrhagic phenotype in patients with von Willebrand disease (VWD) is heterogeneous, and assays of von Willebrand factor ristocetin cofactor activity (VWF:RCo) do not always reflect clinical severity, especially in those individuals classed as type 1 VWD. Recent studies have shown that whole blood ristocetin-induced platelet agglutination (WB-RIPA) using an easy-to-use analyzer, Multiplate® platelet impedance technique, could be informative as a diagnostic test in VWD, although inconsistencies were evident in patients with the type 1 disorder, possibly associated with clinical symptoms.

Aim: To investigate the relationship between WB-RIPA, bleeding scores (BS) and VWF-related measurements in type 1 VWD. Read More

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http://dx.doi.org/10.1111/hae.13725DOI Listing

Endothelial to Mesenchymal Transition: Role in Physiology and in the Pathogenesis of Human Diseases.

Physiol Rev 2019 Apr;99(2):1281-1324

Jefferson Institute of Molecular Medicine, Thomas Jefferson University , Philadelphia, Pennsylvania.

Numerous studies have demonstrated that endothelial cells are capable of undergoing endothelial to mesenchymal transition (EndMT), a newly recognized type of cellular transdifferentiation. EndMT is a complex biological process in which endothelial cells adopt a mesenchymal phenotype displaying typical mesenchymal cell morphology and functions, including the acquisition of cellular motility and contractile properties. Endothelial cells undergoing EndMT lose the expression of endothelial cell-specific proteins such as CD31/platelet-endothelial cell adhesion molecule, von Willebrand factor, and vascular-endothelial cadherin and initiate the expression of mesenchymal cell-specific genes and the production of their encoded proteins including α-smooth muscle actin, extra domain A fibronectin, N-cadherin, vimentin, fibroblast specific protein-1, also known as S100A4 protein, and fibrillar type I and type III collagens. Read More

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http://dx.doi.org/10.1152/physrev.00021.2018DOI Listing
April 2019
1 Read

In vitro phosphorylation of von Willebrand factor by FAM20c enhances its ability to support platelet adhesion.

J Thromb Haemost 2019 Mar 12. Epub 2019 Mar 12.

Department of Medicine, Baylor College of Medicine.

Background: von Willebrand factor (VWF) mediates platelet adhesion and contributes to hemostasis at sites of vascular injury as well as to arterial thrombosis. The A1A2A3 domains of VWF contain important sites that differentially participate in supporting platelet adhesion. FAM20c (family with sequence similarity 20, member C) has emerged as a serine/threonine kinase, which phosphorylates extracellular proteins containing the S-X-E/pS motifs that are also found within the VWF A domains. Read More

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http://dx.doi.org/10.1111/jth.14426DOI Listing

Desialylation of platelets induced by Von Willebrand Factor is a novel mechanism of platelet clearance in dengue.

PLoS Pathog 2019 Mar 8;15(3):e1007500. Epub 2019 Mar 8.

Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.

Thrombocytopenia and platelet dysfunction are commonly observed in patients with dengue virus (DENV) infection and may contribute to complications such as bleeding and plasma leakage. The etiology of dengue-associated thrombocytopenia is multifactorial and includes increased platelet clearance. The binding of the coagulation protein von Willebrand factor (VWF) to the platelet membrane and removal of sialic acid (desialylation) are two well-known mechanisms of platelet clearance, but whether these conditions also contribute to thrombocytopenia in dengue infection is unknown. Read More

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http://dx.doi.org/10.1371/journal.ppat.1007500DOI Listing
March 2019
1 Read

Methodology for the establishment of primary porcine vocal fold epithelial cell cultures.

Laryngoscope 2019 Mar 8. Epub 2019 Mar 8.

Department of Surgery, Division of Otolaryngology-Head & Neck Surgery, University of Wisconsin-Madison, Madison, Wisconsin, U.S.A.

Objective: A current lack of methods for epithelial cell culture significantly hinders our understanding of the role of the epithelial and mucus barriers in vocal fold health and disease. Our first objective was to establish reproducible techniques for the isolation and culture of primary porcine vocal fold epithelial cells. Our second objective was to evaluate the functional significance of cell cultures using an in vitro exposure to an inflammatory cytokine. Read More

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http://doi.wiley.com/10.1002/lary.27909
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http://dx.doi.org/10.1002/lary.27909DOI Listing
March 2019
2 Reads

von Willebrand Factor and Management of Heart Valve Disease: JACC Review Topic of the Week.

J Am Coll Cardiol 2019 Mar;73(9):1078-1088

CHU Lille, Université de Lille, Inserm U1011 - EGID, Institut Pasteur de Lille, Lille, France; CHU Lille, Hematology and Transfusion, Lille, France. Electronic address:

For decades, numerous observations have shown an intimate relationship between von Willebrand factor (VWF) multimer profile and heart valve diseases (HVD). The current knowledge of the unique biophysical properties of VWF helps us to understand the longstanding observations concerning the bleeding complications in patients with severe HVD. Not only does the analysis of the VWF multimer profile provide an excellent evaluation of HVD severity, it is also a strong predictor of clinical events. Read More

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http://dx.doi.org/10.1016/j.jacc.2018.12.045DOI Listing
March 2019
1 Read

Plasma Levels of Vascular Endothelial Growth Factor and Selected Hemostatic Parameters in Association With Treatment Response in Multiple Myeloma.

Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029618823280

1 Department of Haematology and Transfusion Medicine, National Centre of Haemostasis and Thrombosis, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Kollarova, Martin, Slovakia.

Multiple myeloma (MM) is a neoplastic plasma cell disorder characterized by the clonal proliferation of plasma cells in the bone marrow and presence of monoclonal protein in the blood or urine. Diverse hemostatic abnormalities have been reported in patients with myeloma which predispose the patient to bleeding and also thrombosis. The aim of this study was to measure the concentrations of serum levels of vascular endothelial growth factor, D-dimer, and von Willebrand factor in patients with newly diagnosed or relapsed multiple myeloma before treatment, during therapy, and after successful therapy. Read More

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http://dx.doi.org/10.1177/1076029618823280DOI Listing
March 2019
2 Reads

Platelets compensate for poor thrombin generation in type 3 von Willebrand disease.

Platelets 2019 Mar 5:1-9. Epub 2019 Mar 5.

b Faculty of Medicine , University of Helsinki , Helsinki , Finland.

In type 3 von Willebrand disease (VWD3), the most severe form with absent von Willebrand factor (VWF), the bleeding phenotype is variable. Platelet contribution to the hemostatic defect in VWD3 calls upon further studies. We investigated the contribution of platelets to in vitro thrombin generation (TG) and platelet procoagulant activity in VWD3. Read More

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http://dx.doi.org/10.1080/09537104.2019.1581922DOI Listing
March 2019
2 Reads

In Vitro Measurement and Modeling of Platelet Adhesion on VWF-Coated Surfaces in Channel Flow.

Biophys J 2019 Feb 8. Epub 2019 Feb 8.

Chemical Engineering, Stanford University, Stanford, California; Mechanical Engineering, Stanford University, Stanford, California; Institute for Computational and Mathmatical Engineering, Stanford University, Stanford, California.

The process of platelet adhesion is initiated by glycoprotein (GP)Ib and GPIIbIIIa receptors on the platelet surface binding with von Willebrand factor on the vascular walls. This initial adhesion and detachment of a single platelet is a complex process that involves multiple bonds forming and breaking and is strongly influenced by the surrounding blood-flow environment. In addition to bond-level kinetics, external factors such as shear rate, hematocrit, and GPIb and GPIIbIIIa receptor densities have also been identified as influencing the platelet-level rate constants in separate studies, but this still leaves a gap in understanding between these two length scales. Read More

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http://dx.doi.org/10.1016/j.bpj.2019.01.040DOI Listing
February 2019

Beneficial Effects of Mesenchymal Stem Cell Delivery via a Novel Cardiac Bioscaffold on Right Ventricles of Pulmonary Arterial Hypertensive Rats.

Am J Physiol Heart Circ Physiol 2019 Mar 1. Epub 2019 Mar 1.

Mechanical Engineering, Colorado State University, United States.

Right ventricular failure (RVF) is a common cause of death in patients suffering from pulmonary arterial hypertension (PAH). The current treatment for PAH only moderately improves symptoms and RVF ultimately occurs. Therefore, it is necessary to develop new treatment strategies to protect against RV maladaptation despite PAH progression. Read More

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http://dx.doi.org/10.1152/ajpheart.00091.2018DOI Listing

Relevance of platelet desialylation and thrombocytopenia in type 2B von Willebrand disease: preclinical and clinical evidence.

Haematologica 2019 Feb 28. Epub 2019 Feb 28.

INSERM;

Patients with type 2B von Willebrand disease (caused by gain-of-function mutations in the gene coding for von Willebrand factor) display bleeding to a variable extent and, in some cases, thrombocytopenia. There are several underlying causes of thrombocytopenia in type 2B von Willebrand disease. It was recently suggested that desialylation-mediated platelet clearance leads to thrombocytopenia in this disease. Read More

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http://dx.doi.org/10.3324/haematol.2018.206250DOI Listing
February 2019

Von Willebrand disease screening in women undergoing hysterectomy for heavy menstrual bleeding.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital/The Ohio State University, Columbus, Ohio.

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http://dx.doi.org/10.1111/hae.13709DOI Listing
February 2019

Genetic variants of VWF gene in type 2 von Willebrand disease.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Fundação HEMOMINAS, Minas Gerais, Brazil.

Introduction: von Willebrand disease (VWD) is the most common inherited bleeding disorder. Few studies have explored the molecular basis of type 2 VWD.

Aim: This study aimed to identify variants associated with type 2 VWD. Read More

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http://dx.doi.org/10.1111/hae.13714DOI Listing
February 2019
1 Read

A case off TTP temporally associated with cocaine use: Implications for treatment of suspected DITMA.

J Clin Apher 2019 Feb 18. Epub 2019 Feb 18.

Department of Medicine, West Virginia University Hospital, Morgantown, West Virginia.

Thrombotic microangiopathies are rare diseases associated with significant morbidity and mortality. The treatment of thrombotic thrombocytopenic purpura (TTP) and drug-induced thrombotic microangiopathy (DITMA) remains a diagnostic dilemma as they present similarly but respond differently to standard treatment with plasma exchange. TTP is a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 resulting in von Willebrand factor aggregates. Read More

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http://doi.wiley.com/10.1002/jca.21687
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http://dx.doi.org/10.1002/jca.21687DOI Listing
February 2019
4 Reads

A Two-Month-Old Child with Vascular Ectasia: A Case Report Diagnosed by Molecular Karyotyping.

J Pediatr Genet 2019 Mar 22;8(1):20-23. Epub 2018 Aug 22.

Department of Pathology, Çukurova Üniversity, Balcali Hospital, Adana, Turkey.

Gastrointestinal angiodysplasia can be encountered in cases with aortic stenosis, inflammatory gastrointestinal conditions, von Willebrand disease or vascular damage, and degenerative changes. Predisposing factors have been described in four adults with vascular ectasia located in the stomach, duodenum, and the distal esophagus. Here, we report a 2-month-old infant with vascular ectasia in the proximal esophagus and diagnosed by molecular karyotyping. Read More

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http://dx.doi.org/10.1055/s-0038-1669385DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375715PMC
March 2019
1 Read

Inherited Bleeding Disorders in Iraq and Consanguineous Marriage.

Int J Hematol Oncol Stem Cell Res 2018 Oct;12(4):273-281

The National Center of Hematology, Al-Mustansiriyah University, Baghdad, Iraq.

Consanguineous marriage is defined as inbreeding between second cousins or closer. In such families there will be a potential increase in the autosomal recessive traits with its lethal effect, with an increased risk of morbidity and mortality in the new generation. Inherited bleeding disorders (InBDs) are rare complicated diseases, difficult and expensive to treat, the defect usually due to quantitative or qualitative deficiency of clotting factors, platelets or fibrinolysis. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375379PMC
October 2018
1 Read

Enhanced processing of von Willebrand factor reflects disease severity and discriminates severe portal hypertension in cirrhosis.

Eur J Gastroenterol Hepatol 2019 Feb 12. Epub 2019 Feb 12.

Clinical Physiology, Center of Functional Imaging and Research, Faculty of Health Sciences, Hvidovre Hospital, University of Copenhagen, Copenhagen, Denmark.

Objectives: Portal hypertension in cirrhosis is associated with endothelial dysfunction, impaired wound healing, and decreased platelet count. Increased von Willebrand factor (VWF) formation has been suggested as a compensatory mechanism, but the role of VWF processing has not been directly assessed. The aim was to measure the processing of activated VWF (VWF-A) in addition to VWF release (VWF-N) to investigate the association of primary hemostasis with disease activity and portal hypertension in liver cirrhosis. Read More

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http://dx.doi.org/10.1097/MEG.0000000000001380DOI Listing
February 2019
1 Read

Case report of two siblings with type 2A von Willebrand disease involving a novel mutation within the calcium-binding site of the A2 domain of von Willebrand factor.

Blood Coagul Fibrinolysis 2019 Feb 11. Epub 2019 Feb 11.

Department of Hematology and Oncology, Texas Tech University Health Sciences Center, Lubbock, Texas.

: Calcium-binding at the A2 domain protects von Willebrand factor (VWF) from cleavage by a disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS13) and is coordinated by five important residues (p.Asp1596, p.Arg1597, p. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000798DOI Listing
February 2019

Restenosis is associated with prothrombotic plasma fibrin clot characteristics in endovascularly treated patients with critical limb ischemia.

J Thromb Thrombolysis 2019 Feb 14. Epub 2019 Feb 14.

Institute of Cardiology, Jagiellonian University Medical College, Krakow, Poland.

Introduction: Hypolysible fibrin clots composed of tightly packed fibers characterize patients with peripheral artery disease (PAD) especially those with critical limb ischemia (CLI). Little is known about the impact of a prothrombotic clot phenotype on restenosis following endovascular revascularization in CLI. The goal of this study was to compare fibrin clot properties and their determinants in CLI patients with restenosis after endovascular treatment (ET) and those free of this complication. Read More

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http://dx.doi.org/10.1007/s11239-019-01826-9DOI Listing
February 2019
2 Reads

Current Trend of Invasive Orthopaedic Interventions for People with Haemophilia in Romania: Single Centre Experience.

Hamostaseologie 2019 Feb 13. Epub 2019 Feb 13.

Department of Pediatric Hemato-Oncology, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania.

Objective:  In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years.

Patients And Methods:  This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. Read More

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http://dx.doi.org/10.1055/s-0039-1677884DOI Listing
February 2019
1 Read

You Can't Heyde Forever.

Eur J Case Rep Intern Med 2018 27;5(9):000896. Epub 2018 Sep 27.

Internal Medicine Service 1.2, Hospital de São José, Centro Hospitalar Lisboa Central, E.P.E - Lisboa, Portugal.

Heyde's syndrome describes an association between aortic stenosis and a predisposition to bleeding from intestinal angiodysplasia resulting from acquired von Willebrand disease. We present the case of an 82-year-old woman with recurrent intestinal bleeding, severe anaemia and secondary myocardial infarction. Investigation identified ileal angiectasia as the source of haemorrhage. Read More

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http://dx.doi.org/10.12890/2018_000896DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346819PMC
September 2018
1 Read

Low endoscopy bleeding risk in patients with congenital bleeding disorders.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Division of Gastroenterology, McGill University Health Centre, Montreal, Quebec, Canada.

Introduction: Haemophilia A and haemophilia B, von Willebrand disease (VWD), factor VII deficiency and factor XI deficiency are congenital bleeding disorders predisposing to bleeding during invasive procedures. The ageing population of people with congenital bleeding disorders will likely increasingly require gastrointestinal endoscopy. The bleeding risk postgastrointestinal endoscopy and optimal prophylactic treatment regimens are not well described. Read More

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http://doi.wiley.com/10.1111/hae.13691
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http://dx.doi.org/10.1111/hae.13691DOI Listing
February 2019
11 Reads

Contraceptive Needs of Adolescents with Chronic Illness.

Pediatr Ann 2019 Feb;48(2):e78-e85

Contraception and sexual health form a key part of comprehensive health care for all adolescents, including those who suffer from chronic illness. Multiple studies have shown that adolescents with chronic illness have rates of sexual activity equal to or greater than their healthy counterparts. Primary care pediatricians have the most comprehensive view of the health of their medically complex patients and the benefit of a longstanding relationship. Read More

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http://dx.doi.org/10.3928/19382359-20190121-01DOI Listing
February 2019
1 Read

A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF/FVIII concentrates in severe inherited von Willebrand disease.

Blood Transfus 2019 Feb 4:1-8. Epub 2019 Feb 4.

Division of Haematology and Transfusion Medicine, "Luigi Sacco" University Hospital, Milan, Italy.

Background: There is a lack of prospective clinical trials specifically designed to evaluate the benefits of prophylaxis with vWF/FVIII concentrates in patients with inherited von Willebrand disease (vWD). The aim of the study was to compare efficacy of secondary long-term prophylaxis (PRO) with vWF/FVIII in the prevention of bleeding episodes in severe vWD patients to standard of care (on-demand treatment; ODT).

Materials And Methods: In this 12-month, phase III, open-label study (PRO. Read More

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http://dx.doi.org/10.2450/2019.0183-18DOI Listing
February 2019
2 Reads

Hypercoagulability and Risk of Venous Thromboembolic Events in Endogenous Cushing's Syndrome: A Systematic Meta-Analysis.

Front Endocrinol (Lausanne) 2018 28;9:805. Epub 2019 Jan 28.

Northwest Pituitary Center, Oregon Health & Science University, Portland, OR, United States.

Hypercortisolism has been implicated in the development of venous thromboembolic events (VTE). We aimed to characterize VTE risk in endogenous Cushing's syndrome (CS) patients, compare that risk to other pathologies, and determine if there are any associated coagulation factor changes. Medline and Scopus search for "hypercortisolism" and "thromboembolic disease" from January 1980 to April 2017 to include studies that reported VTE rates and/or coagulation profile of CS patients. Read More

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http://dx.doi.org/10.3389/fendo.2018.00805DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360168PMC
January 2019
1 Read

Quantitative analysis of desmopressin (DDAVP) response in adult patients with type 1 von Willebrand disease.

Int J Lab Hematol 2019 Feb 8. Epub 2019 Feb 8.

Division of Hematology, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts.

Introduction: Patients with Type 1 von Willebrand disease (VWD) have reduced amounts of von Willebrand factor (VWF) in their blood. Desmopressin (DDAVP) has been used to raise the blood levels of VWF in these patients. However, not all patients with Type 1 VWD are known to respond to DDAVP therapy. Read More

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http://dx.doi.org/10.1111/ijlh.12978DOI Listing
February 2019
1 Read

Optimization of von Willebrand factor multimer analysis in vertical mini-gel electrophoresis systems: A rapid procedure.

Thromb Res 2019 Mar 30;175:76-83. Epub 2019 Jan 30.

Instituto Butantan, Laboratório de Fisiopatologia, São Paulo, SP, Brazil; Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. Electronic address:

Von Willebrand disease (VWD) is a common cause of bleeding worldwide. Analysis of von Willebrand factor (VWF) multimer distribution (VWF:MD) is essential to properly classify and treat different types of VWD, and it is performed using a SDS agarose gel electrophoresis followed by Western blotting, a handmade technique that demands days to be completed and requires skillful execution. Aiming both to facilitate gel production and to shorten the preparation time, we developed an uncomplicated technique to provide agility in the analysis of VWF:MD, so that it can be easily accomplished in the routine practice of hemostasis laboratories. Read More

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http://dx.doi.org/10.1016/j.thromres.2019.01.018DOI Listing
March 2019
2 Reads

An Inhibitory Effect of Dryocrassin ABBA on vWbp That Protects Mice From Pneumonia.

Front Microbiol 2019 23;10. Epub 2019 Jan 23.

College of Animal Sciences, Jilin University, Changchun, China.

Von Willebrand factor-binding protein (vWbp), secreted by (), can activate host prothrombin, convert fibrinogen to fibrin clots, induce blood clotting, and contribute to pathophysiology of -related diseases, including infective endocarditis, staphylococcal sepsis and pneumonia. Therefore, vWbp is an promising drug target in the treatment of related infections. Here, we report that dryocrassin ABBA (ABBA), a natural compound derived from , can significantly inhibit the coagulase activity of vWbp by directly interacting with vWbp without killing the bacteria or inhibiting the expression of the vWbp. Read More

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https://www.frontiersin.org/article/10.3389/fmicb.2019.00007
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http://dx.doi.org/10.3389/fmicb.2019.00007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351477PMC
January 2019
4 Reads

A simple protocol for isolating mouse lung endothelial cells.

Sci Rep 2019 Feb 6;9(1):1458. Epub 2019 Feb 6.

Department of Medicine, Aab Cardiovascular Research Institute, University of Rochester, Rochester, NY, 14623, USA.

Endothelial dysfunction is the common molecular basis of multiple human diseases, such as atherosclerosis, diabetes, hypertension, and acute lung injury. Therefore, primary isolation of high-purity endothelial cells (ECs) is crucial to study the mechanisms of endothelial function and disease pathogenesis. Mouse lung ECs (MLECs) are widely used in vascular biology and lung cell biology studies such as pulmonary inflammation, angiogenesis, vessel permeability, leukocyte/EC interaction, nitric oxide production, and mechanotransduction. Read More

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http://dx.doi.org/10.1038/s41598-018-37130-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365507PMC
February 2019
7 Reads

[Laboratory signs of activation of the hemostasis system in patients with coronary heart disease, who had coronary artery bypass surgery.]

Klin Lab Diagn 2018 ;63(8):500-505

Federal State Budgetary Educational Institution of Higher Education "Omsk State Medical University" of the Ministry of Health. 644000, Omsk, Russia.

The hypercoagulable state is a risk factor for shunt thrombosis in patients who had coronary artery bypass surgery. That is why the study of factors and operative intervention which can lead to hypercoagulable states are extremely valuable and vital issues. 85 patients who had coronary artery bypass surgery were examined. Read More

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http://dx.doi.org/10.18821/0869-2084-2018-63-8-500-505DOI Listing
January 2018
4 Reads

Analysis of von Willebrand Disease in the South Moravian Population (Czech Republic): Results from the BRNO-VWD Study.

Thromb Haemost 2019 Apr 5;119(4):594-605. Epub 2019 Feb 5.

Haemostasis Unit, Antwerp University Hospital, Edegem, Belgium.

Background:  von Willebrand disease (VWD) is an inherited bleeding disorder caused by a quantitative (type 1 and 3) or qualitative (type 2) defect of von Willebrand factor (VWF). The heterogeneity of laboratory phenotyping makes diagnosing difficult.

Objective:  A cross-sectional, family-based VWD study in a collaboration between University Hospital Brno (Czech Republic) and Antwerp University Hospital (Belgium) to improve the understanding of laboratory phenotype/genotype correlation. Read More

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http://dx.doi.org/10.1055/s-0039-1678528DOI Listing
April 2019
1 Read

High On-Treatment Platelet Reactivity Determinants on Dual Antiplatelet Therapy in Patients With Ischemic Heart Disease Before Elective Percutaneous Coronary Intervention.

Kardiologiia 2018 Nov 18;58(4):5-14. Epub 2018 Nov 18.

ФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии им. А. Н. Бакулева» Минздрава России.

Objective: to determine impact of different laboratory and genetic factors on high on-treatment platelet reactivity (HOPR) during dual antiplatelet therapy (DAPT).

Methods: We included in this study 94 patients with stable ischemic heart disease (mean age 59±9.67 years). Read More

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http://dx.doi.org/10.18087/cardio.2018.4.10104DOI Listing
November 2018
2 Reads

Factor VIII and vWF deficiency in STT3A-CDG.

J Inherit Metab Dis 2019 Mar 30;42(2):325-332. Epub 2019 Jan 30.

Department of Pediatrics, Division of Medical Genetics, University of Washington, Seattle, Western Australia.

STT3A-CDG (OMIM# 615596) is an autosomal recessive N-linked glycosylation disorder characterized by seizures, developmental delay, intellectual disability, and a type I carbohydrate deficient transferrin pattern. All previously reported cases (n = 6) have been attributed to a homozygous pathogenic missense variant c.1877C>T (p. Read More

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http://dx.doi.org/10.1002/jimd.12021DOI Listing
March 2019
1 Read
3.365 Impact Factor

Exploring the "minimal" structure of a functional ADAMTS13 by mutagenesis and small-angle X-ray scattering.

Blood 2019 Jan 28. Epub 2019 Jan 28.

Departments of Medicine, Biochemistry and Molecular Biophysics, Washington University School of Medicine, St. Louis, MO, United States.

Human ADAMTS13 is a multidomain protein with metalloprotease (M), disintegrin-like (D), thrombospondin-1 (T), Cys-rich (C) and spacer (S) domains, followed by 7 additional T and 2 CUB domains. ADAMTS13 inhibits the growth of von Willebrand factor (VWF)-platelet aggregates by cleaving the cryptic Tyr-Met bond in the VWF A2 domain. ADAMTS13 is regulated by substrate-induced allosteric activation: without shear stress, the distal T8-CUB domains markedly inhibits VWF cleavage, and binding of VWF domain D4 or selected monoclonal antibodies (MAbs) to distal ADAMTS13 domains relieves this autoinhibition. Read More

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http://dx.doi.org/10.1182/blood-2018-11-886309DOI Listing
January 2019
1 Read

Challenges in diagnosis of von Willebrand disease in the presence of combined mutations of different genes.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Centre d'hémostase pédiatrique et adulte du CHU Sainte-Justine, Montreal University, Montréal, Quebec, Canada.

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http://dx.doi.org/10.1111/hae.13686DOI Listing
January 2019
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Thrombotic complications in adult patients with severe single coagulation factor or platelet defects - an overview.

Expert Rev Hematol 2019 Feb 28;12(2):119-128. Epub 2019 Jan 28.

a Section for Hematology, Department of Clinical Science , University of Bergen , Bergen , Norway.

Introduction: Even though thrombotic events are rare in patients with coagulation deficiencies, several cases of both arterial and venous thromboses have been reported in patients with single coagulation factor defects and platelet defects. Thromboses have been described both in hemophilia A and B, von Willebrand disease as well as in many other rare congenital coagulation factor and platelet defects. Thromboses may also occur in patients with acquired hemophilia and in patients with severe thrombocytopenia due to hematological malignancies or intensive chemotherapy. Read More

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http://dx.doi.org/10.1080/17474086.2019.1570126DOI Listing
February 2019
4 Reads

Unfavourably altered plasma clot properties in patients with primary Raynaud's phenomenon: association with venous thromboembolism.

J Thromb Thrombolysis 2019 Feb;47(2):248-254

John Paul II Hospital, Krakow, Poland.

Associations of Raynaud's phenomenon (RP) with venous thromboembolism (VTE) are unclear. We investigated the occurrence of RP together with prothrombotic state markers and fibrin clot properties in VTE patients. In this prospective cohort study we enrolled 360 patients free of known autoimmune disease. Read More

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http://dx.doi.org/10.1007/s11239-019-01805-0DOI Listing
February 2019
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Regulation of von Willebrand Factor Gene in Endothelial Cells that Are Programmed to Pluripotency and Differentiated Back to Endothelial Cells.

Stem Cells 2019 Jan 25. Epub 2019 Jan 25.

Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.

Endothelial cells play a central role in physiological function and pathophysiology of blood vessels in health and disease. However, the molecular mechanism that establishes the endothelial phenotype, and contributes to its signature cell type-specific gene expression, is not yet understood. We studied the regulation of a highly endothelial-specific gene, von Willebrand factor (VWF), in induced pluripotent stem cells generated from primary endothelial cells (human umbilical vein endothelial cells [HUVEC] into a pluripotent state [HiPS]) and subsequently differentiated back into endothelial cells. Read More

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http://dx.doi.org/10.1002/stem.2978DOI Listing
January 2019
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Biomarkers for Acute Respiratory Distress syndrome and prospects for personalised medicine.

J Inflamm (Lond) 2019 15;16. Epub 2019 Jan 15.

1Department of Morphology, Surgery and Experimental Medicine, Intensive Care Section, University of Ferrara, 44121 Ferrara, Italy.

Acute lung injury (ALI) affects over 10% of patients hospitalised in critical care, with acute respiratory distress syndrome (ARDS) being the most severe form of ALI and having a mortality rate in the region of 40%. There has been slow but incremental progress in identification of biomarkers that contribute to the pathophysiology of ARDS, have utility in diagnosis and monitoring, and that are potential therapeutic targets (Calfee CS, Delucchi K, Parsons PE, Thompson BT, Ware LB, Matthay MA, Thompson T, Ware LB, Matthay MA, Lancet Respir Med 2014, 2:611--620). However, a major issue is that ARDS is such a heterogeneous, multi-factorial, end-stage condition that the strategies for "lumping and splitting" are critical (Prescott HC, Calfee CS, Thompson BT, Angus DC, Liu VX, Am J Respir Crit Care Med 2016, 194:147--155). Read More

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http://dx.doi.org/10.1186/s12950-018-0202-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332898PMC
January 2019
3 Reads
2.024 Impact Factor

Impaired functional recovery of endothelial colony-forming cells from moyamoya disease in a chronic cerebral hypoperfusion rat model.

J Neurosurg Pediatr 2018 Nov 1:1-10. Epub 2018 Nov 1.

1Division of Pediatric Neurosurgery, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital.

OBJECTIVEEndothelial colony-forming cells (ECFCs) isolated from pediatric patients with moyamoya disease (MMD) have demonstrated decreased numbers and defective functioning in in vitro experiments. However, the function of ECFCs has not been evaluated using in vivo animal models. In this study, the authors compared normal and MMD ECFCs using a chronic cerebral hypoperfusion (CCH) rat model. Read More

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http://dx.doi.org/10.3171/2018.8.PEDS1883DOI Listing
November 2018
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Targeting the perivascular niche sensitizes disseminated tumour cells to chemotherapy.

Nat Cell Biol 2019 Feb 21;21(2):238-250. Epub 2019 Jan 21.

Public Health Sciences Division/Translational Research Program, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.

The presence of disseminated tumour cells (DTCs) in bone marrow is predictive of poor metastasis-free survival of patients with breast cancer with localized disease. DTCs persist in distant tissues despite systemic administration of adjuvant chemotherapy. Many assume that this is because the majority of DTCs are quiescent. Read More

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http://www.nature.com/articles/s41556-018-0267-0
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http://dx.doi.org/10.1038/s41556-018-0267-0DOI Listing
February 2019
18 Reads
19.679 Impact Factor

Evaluation of von Willebrand factor concentrates by platelet adhesion to collagen using an in vitro flow assay.

Res Pract Thromb Haemost 2019 Jan 7;3(1):126-135. Epub 2018 Dec 7.

Department of Haematology Imperial College of Science Technology and Medicine London UK.

Background: Von Willebrand disease (VWD) results from quantitative or qualitative deficiency of von Willebrand factor (VWF) and is treated using VWF-containing concentrates. Several studies have compared the function of various VWF containing concentrates however this has not been performed using shear based assays.

Objectives: To compare the platelet-capture potential of 10 commercially available, plasma-derived VWF concentrates under shear conditions. Read More

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http://dx.doi.org/10.1002/rth2.12166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332833PMC
January 2019
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Education needs of nurses in thrombosis and hemostasis: An international, mixed-methods study.

Res Pract Thromb Haemost 2019 Jan 1;3(1):99-108. Epub 2018 Nov 1.

Nursing Research Royal Children's Hospital Melbourne Victoria Australia.

Background: The need for a more integrated, multidisciplinary approach to care for individuals with bleeding or clotting disorders has been highlighted in recent years. Evidence-based education adapted to nurses' needs is essential for a successful evolution. However, limited data currently exist on the clinical challenges nurses face in this specialty area. Read More

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http://doi.wiley.com/10.1002/rth2.12155
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http://dx.doi.org/10.1002/rth2.12155DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332710PMC
January 2019
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Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine.

Res Pract Thromb Haemost 2019 Jan 16;3(1):26-37. Epub 2018 Nov 16.

Departments of Epidemiology & Biostatistics, Medicine University of Oklahoma Health Sciences Center Oklahoma City Oklahoma.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by severe congenital or immune-mediated deficiency in ADAMTS13, the enzyme that cleaves von Willebrand factor multimers. This rare condition leads invariably and rapidly to a fatal outcome in the absence of treatment, and therefore raises multiple diagnostic and therapeutic challenges. The novel concepts and mechanisms identified in the laboratory for this disease have been rapidly and successfully translated into the clinic for the benefit of patients, making TTP an archetypal disease that has benefited from targeted therapies. Read More

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http://dx.doi.org/10.1002/rth2.12160DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332733PMC
January 2019
3 Reads

Mechanisms of thrombocytopenia in platelet-type Von Willebrand Disease.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Department of Medicine, University of Perugia;

Platelet-type von Willebrand disease is an inherited platelet disorder characterized by thrombocytopenia with large platelets caused by gain-of-function variants in GP1BA leading to enhanced GPIbα von Willebrand factor interaction. GPIbα and von Willebrand factor play a role in megakaryocytopoiesis, thus we aimed to investigate megakaryocyte differentiation and proplatelet-formation in platelet-type von Willebrand disease using megakaryocytes from a patient carrying the Met239Val variant and from mice carrying the Gly233Val variant. Platelet-type von Willebrand disease megakaryocytes bound von Willebrand factor at an early differentiation stage and generated proplatelets with a decreased number of enlarged tips as compared with control megakaryocytes. Read More

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http://dx.doi.org/10.3324/haematol.2018.200378DOI Listing
January 2019
3 Reads