12,803 results match your criteria von Willebrand Disease

Characterization of the human ABO genotypes and their association to common inflammatory and cardiovascular disease in the UK Biobank.

Am J Hematol 2021 Jul 30. Epub 2021 Jul 30.

Department of Immunology, Genetics and Pathology, Science for Life Laboratory, Uppsala University, Uppsala, Sweden.

Background: The ABO gene contains three major alleles that encodes different antigens; A, B, and O, which determine an individual's blood group. Previous studies have primarily focused on identifying associations between ABO blood groups and diseases risk. Here, we sought to test for association between ABO genotypes (OO, OA, AA; OB, BB, and AB) and a large set of common inflammatory and cardiovascular diseases in UK Biobank as well as disease-related protein biomarkers in NSPHS. Read More

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Aortic Valve Stenosis, a Precipitating Factor of Recurrent Bleed in Colonic Angiodysplasia: A Literature Review.

Cureus 2021 Jun 24;13(6):e15903. Epub 2021 Jun 24.

Gastroenterology and Hepatology, Community First Medical Center, Chicago, USA.

Angiodysplasia (also known as angioectasia) is a lesion characterized by abnormal, dilated small blood vessels in the mucosa and submucosal layers of the GI tract. With the estimated low incidence of active GI bleeding from these lesions, angiodysplasia can be challenging to diagnose. The presence of aortic stenosis has increased the recognition rate of angiodysplasia, especially in the elderly. Read More

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Trimetazidine Therapy in Coronary Artery Disease: The Impact on Oxidative Stress, Inflammation, Endothelial Dysfunction, and Long-Term Prognosis.

Am J Ther 2021 Jul 16. Epub 2021 Jul 16.

Department of Medical and Surgical Specialties, Faculty of Medicine, Transilvania University of Brasov, Brasov, Romania; Department of Fundamental, Prophylactic and Clinical Disciplines, Faculty of Medicine, Transilvania University of Brasov, Brasov, Romania; Department of Cardiology, Clinical County Emergency Hospital Brasov, Brasov, Romania; and Faculty of Sociology and Communication, Faculty of Economic Sciences and Business Administration, Transilvania University of Brasov, Brasov, Romania.

Background: In coronary artery disease (CAD), reduction of perfusion in coronary arteries is followed by increases of oxidative stress and decreases of adenosine triphosphate reserve. In this condition, trimetazidine (TMZ), a metabolic anti-ischemic agent, seems to be an ideal therapeutic agent because it increases mitochondrial adenosine triphosphate production.

Study Question: To evaluate the impact of TMZ on oxidative stress, inflammation, endothelial dysfunction, and long-term prognosis in CAD. Read More

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Magnesium lithospermate B ameliorates hypobaric hypoxia-induced pulmonary arterial hypertension by inhibiting endothelial-to-mesenchymal transition and its potential targets.

Biomed Pharmacother 2020 Oct 12;130:110560. Epub 2020 Aug 12.

The First Hospital of Lanzhou University, Lanzhou 730000, China. Electronic address:

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodeling leading to elevation of pulmonary artery pressure, right ventricular hypertrophy, and death. Currently, there are no cure exists for PAH. Magnesium lithospermate B (MLB) is the major component of Salvia przewalskii water extracts with treating angina and cardiovascular damage, anti-inflammation, anti-oxidation and anti-apoptosis. Read More

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October 2020

Coronary Artery Fistula: An Unexpected Cause of Heart Failure in a 58-Year-Old Woman.

JACC Case Rep 2020 Jul 10;2(8):1192-1195. Epub 2020 Jun 10.

Smidt Heart Institute, Cedars Sinai Medical Center, Los Angeles, California.

Coronary artery fistula (CAF) is an abnormal connection between a coronary artery and a heart chamber resulting in left-to-right shunt. A large CAF was an unexpected cause of heart failure in a 58-year-old woman who underwent transcatheter closure of the CAF with improvement in symptoms but complicated by myocardial infarction. (). Read More

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Diversity analysis of genes encoding Mfa1 fimbrial components in Porphyromonas gingivalis strains.

PLoS One 2021 26;16(7):e0255111. Epub 2021 Jul 26.

Department of Microbiology, School of Dentistry, Aichi Gakuin University, Nagoya, Japan.

Porphyromonas gingivalis, a gram-negative anaerobic bacterium, is associated with the development of periodontal disease. The genetic diversity in virulence factors, such as adhesive fimbriae, among its strains affects the bacterial pathogenicity. P. Read More

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Inactivation of Cerebral Cavernous Malformation Genes Results in Accumulation of von Willebrand Factor and Redistribution of Weibel-Palade Bodies in Endothelial Cells.

Front Mol Biosci 2021 9;8:622547. Epub 2021 Jul 9.

Department of Human Genetics, Interfaculty Institute of Genetics and Functional Genomics, University Medicine Greifswald, Greifswald, Germany.

Cerebral cavernous malformations are slow-flow thrombi-containing vessels induced by two-step inactivation of the , or gene within endothelial cells. They predispose to intracerebral bleedings and focal neurological deficits. Our understanding of the cellular and molecular mechanisms that trigger endothelial dysfunction in cavernous malformations is still incomplete. Read More

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Illustrated State-of-the-Art Capsules of the ISTH 2020 Congress.

Res Pract Thromb Haemost 2021 Jul 16;5(5):e12532. Epub 2021 Jul 16.

Transfusion Medicine NHS Blood and Transplant (London) and Barts Health NHS Trust London UK.

This year's Congress of the International Society of Thrombosis and Haemostasis (ISTH) was hosted virtually from Philadelphia July 17-21, 2021. The conference, now held annually, highlighted cutting-edge advances in basic, population and clinical sciences of relevance to the Society. Despite being held virtually, the 2021 congress was of the same scope and quality as an annual meeting held in person. Read More

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Backbone resonance assignments of the A2 domain of mouse von Willebrand factor.

Biomol NMR Assign 2021 Jul 20. Epub 2021 Jul 20.

Department of Molecular Engineering, Graduate School of Engineering, Kyoto University, Kyoto-Daigaku Katsura, Nishikyo-Ku, Kyoto, 615-8510, Japan.

von Willebrand factor (vWF) is an adhesive plasma protein that is important for platelet adhesion in normal hemostasis in response to vascular injury. Although large vWF multimers are released from storage granules of platelets and (sub-)endothelial cells in response to hemostatic stimuli, for normal physiological function, vWF multimers are required to be cleaved into smaller multimeric forms. The plasma metalloproteinase ADAMTS13 specifically cleaves the peptide bond located in the middle of the A2 domain of vWF (vWF-A2), but the cleavage site is buried inside the structure of vWF and is difficult to access in the absence of elevated flow shear stress. Read More

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From Other Journals: A Review of Recent Articles by Our Editorial Team.

Pediatr Cardiol 2021 Jul 20. Epub 2021 Jul 20.

Heart Institute, Johns Hopkins All Children's All Children's Hospital, St. Petersburg, FL, USA.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) outcomes for COVID-19 infection in adults with congenital heart disease which showed no increased mortality compared to the general population, (2) hepatorenal dysfunction before transplantation in patients with Fontan is associated with increased mortality, (3) abnormal Von Willebrand factor metabolism and angiopoietin signaling may contribute to pulmonary AVM formation in children with a Glenn circulation, (4) low cardiac output after the Norwood procedure which improves with higher hemoglobin and with milrionone, (5) a comparison of staged versus complete repair in neonatal tetralogy of Fallot reveiling the pros and cons of each strategy, (6) the long-term outcomes of early repair of complete atrioventricular canal show no difference in outcomes in patients who were repaired below 3 months of life. Read More

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Low-dose immune tolerance induction alone or with immunosuppressants according to prognostic risk factors in Chinese children with hemophilia A inhibitors.

Res Pract Thromb Haemost 2021 Jul 14;5(5):e12562. Epub 2021 Jul 14.

Hemophilia Comprehensive Care Center Hematology Center Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics (Capital Medical University) Key Laboratory of Major Diseases in Children Ministry of Education Beijing Children's Hospital National Center for Children's Health Capital Medical University Beijing China.

Background: In developing countries, children with hemophilia A (HA) with high-titer inhibitor and poor immune tolerance induction (ITI) prognostic risk(s) cannot afford the recommended high- or intermediate-dose ITI.

Objectives: To determine the efficacy of low-dose ITI (plasma-derived factor VIII [FVIII]/von Willebrand factor at 50 FVIII IU/kg every other day) by itself (ITI-alone) or combined with immunosuppressants rituximab and prednisone (ITI-IS) in children with HA with high-titer inhibitor.

Methods: All enrolled patients had pre-ITI inhibitor ≥10 BU. Read More

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Rodenticide (Yellow Phosphorus Poison)-Induced Hepatotoxicity in India: Constraints During Management.

J Clin Exp Hepatol 2021 Jul-Aug;11(4):414-417. Epub 2021 Apr 30.

Department of Hepatology, Sri Ramachandra Institute of Higher Education & Research, Chennai, India.

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ABO blood groups do not predict progression of traumatic intracranial hemorrhage.

J Clin Neurosci 2021 Aug 23;90:345-350. Epub 2021 Jun 23.

Division of Trauma & Acute Care Surgery, Department of Surgery, Tufts Medical Center, 800 Washington St, #4488, Boston, MA 02111, United States. Electronic address:

ABO blood groups are associated with genetically predisposed variations in von Willebrand factor (VWF) resulting in higher risks of thrombotic events in non-O blood types and bleeding complications in blood type O. The role of ABO blood groups in progression of traumatic intracranial hemorrhage (TICH) is unknown. Given statistically lower VWF levels in blood type O in the general population, we hypothesized that blood type O patients have a higher risk of such progression. Read More

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Adolescent With von Willebrand Disease Type 3 Spontaneous Abdominal Hemorrhage.

J Emerg Nurs 2021 Jul;47(4):661-668

An adolescent female classified as unstable with a spontaneous abdominal hemorrhage was transferred to a level 1 pediatric trauma tertiary emergency department. Pertinent medical history included von Willebrand disease type 3, menorrhagia, and obesity. Preparation before patient arrival included mobilization of multidisciplinary medical team experts in hematology, pharmacy, blood bank, radiology, and nursing who provided lifesaving interventions. Read More

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Biomarkers of endothelial dysfunction and outcomes in coronavirus disease 2019 (COVID-19) patients: A systematic review and meta-analysis.

Microvasc Res 2021 Jul 14:104224. Epub 2021 Jul 14.

Faculty of Medicine, Universitas Airlangga, Surabaya 60286, Indonesia. Electronic address:

Background: Several studies have reported that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can directly infect endothelial cells, and endothelial dysfunction is often found in severe cases of coronavirus disease 2019 (COVID-19). To better understand the prognostic values of endothelial dysfunction in COVID-19-associated coagulopathy, we conducted a systematic review and meta-analysis to assess biomarkers of endothelial cells in patients with COVID-19.

Methods: A literature search was conducted on online databases for observational studies evaluating biomarkers of endothelial dysfunction and composite poor outcomes in COVID-19 patients. Read More

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Continuous-infusion von Willebrand factor concentrate is effective for the management of acquired von Willebrand disease.

Blood Adv 2021 07;5(14):2813-2816

Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD.

Acquired von Willebrand disease (aVWD) is a rare disorder associated with a reduction in von Willebrand factor (VWF) activity, leading to increased bleeding risk. Monoclonal gammopathy of undetermined significance (MGUS) is the most common cause of lymphoproliferative disorder-associated aVWD and is caused by accelerated clearance of circulating VWF. Standard VWF replacement protocols for congenital VWD based on intermittent bolus dosing are typically less effective for aVWD because of antibody-mediated clearance. Read More

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Absence of exaggerated pharmacology by recombinant ADAMTS13 in the rat and monkey.

Blood Coagul Fibrinolysis 2021 Jul 15. Epub 2021 Jul 15.

Baxalta Innovations GmbH, A Takeda Company, Vienna, Austria.

Insufficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motif repeats-13) is the cause of thrombotic thrombocytopenic purpura (TTP) and contributes in microangiopathy in sickle cell disease (SCD). Recombinant ADAMTS13 effectively cleaves prothrombotic ultra-large von Willebrand factor (VWF) multimers. It is being tested as replacement therapy for TTP, and at supra-physiologic concentrations, for moderating vaso-occlusive crisis in SCD. Read More

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Pathological characteristics of liver sinusoidal thrombosis in COVID-19 patients:a series of 43 cases.

Hepatol Res 2021 Jul 14. Epub 2021 Jul 14.

Section of Digestive Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.

Aim: COVID-19 is characterized by pneumonia with secondary damage to multiple organs including the liver. Liver injury (elevated ALT and AST) often correlates with disease severity in COVID-19 patients. The aim of this study is to identify pathological microthrombi in COVID-19 patient livers by correlating their morphology with liver injury, and examine hyperfibrinogenemia and von Willebrand factor(vWF) as mechanisms of their formation. Read More

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The hemostatic and thrombotic complications of liver disease.

Eur J Haematol 2021 Jul 14. Epub 2021 Jul 14.

Division of Hematology and Oncology, Oregon Health & Science University, Portland, OR, USA.

Hepatic cirrhosis leads to numerous hematologic derangements resulting in a complex and tenuously rebalanced hemostatic milieu. The utility of common hematologic tests including the INR and aPTT in assessing hemostatic and thrombotic risk in patients with cirrhosis is limited, and consensus on transfusion thresholds and proper management of thrombotic complications continues to evolve. This review summarizes the pathophysiology of key derangements of hemostasis including those of platelets, von Willebrand factor, pro- and anticoagulation factors, and fibrin. Read More

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Phage display broadly identifies inhibitor-reactive regions in von Willebrand factor.

J Thromb Haemost 2021 Jul 13. Epub 2021 Jul 13.

Life Sciences Institute, University of Michigan, University of Michigan Medical School, Ann Arbor, MI, USA.

Background: Correction of von Willebrand factor (VWF) deficiency with replacement products containing VWF can lead to the development of anti-VWF alloantibodies (i.e., VWF inhibitors) in patients with severe von Willebrand disease (VWD). Read More

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Autoimmune- and complement-mediated hematologic condition recrudescence following SARS-CoV-2 vaccination.

Blood Adv 2021 07;5(13):2794-2798

Division of Hematology, University of Washington, Seattle, WA.

A variety of autoimmune disorders have been reported after viral illnesses and specific vaccinations. Cases of de novo immune thrombocytopenia (ITP) have been reported after SARS-CoV-2 vaccination, although its effect on preexisting ITP has not been well characterized. In addition, although COVID-19 has been associated with complement dysregulation, the effect of SARS-CoV-2 vaccination on preexisting complementopathies is poorly understood. Read More

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von Willebrand factor variants in C3 glomerulopathy: A Chinese cohort study.

Clin Immunol 2021 Aug 8;229:108794. Epub 2021 Jul 8.

Renal Division, Department of Medicine, Peking University First Hospital; Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China; Beijing 100034, PR China; Peking-Tsinghua Center for Life Sciences, PR China.

C3 glomerulopathy (C3G) is a rare renal disease characterized by predominant glomerular C3 staining. Complement alternative pathway dysregulation due to inherited complement defects is associated with C3G. To identify novel C3G-related genes, we screened 86 genes in the complement, coagulation and endothelial systems in 35 C3G patients by targeted genomic enrichment and massively parallel sequencing. Read More

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Real-World Data on the Effectiveness and Safety of wilate for the Treatment of von Willebrand Disease.

TH Open 2021 Jul 4;5(3):e264-e272. Epub 2021 Jul 4.

Werlhof-Institut GmbH, Hannover, Germany.

 The efficacy and safety of wilate (human von Willebrand factor/coagulation factor VIII) in patients with von Willebrand disease (VWD) has been demonstrated in clinical trials. Here, we present real-world data on the use of wilate for the routine care of patients with VWD.  The objectives of this observational, prospective, phase 4 study were to evaluate the safety, tolerability, and effectiveness of wilate in on-demand treatment of bleeding episodes (BEs), long-term prophylaxis, and surgical prophylaxis among patients with any type of VWD. Read More

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Endothelium Activation Markers in Severe Hospitalized COVID-19 Patients: Role in Mortality Risk Prediction.

TH Open 2021 Jul 4;5(3):e253-e263. Epub 2021 Jul 4.

Department of Immunohematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy.

 Endothelial damage and hypercoagulability are major players behind the hemostatic derangement of SARS-CoV-2 infection.  In this prospective study we assessed endothelial and inflammatory biomarkers in a cohort of COVID-19 patients, aiming to identify predictive factors of in-hospital mortality.  COVID-19 patients hospitalized in intensive care (ICU) and non-ICU units at 2 Bergamo (Italy) hospitals from March 23 to May 30, 2020, were enrolled. Read More

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Mechanisms of immunothrombosis in COVID-19.

Curr Opin Hematol 2021 Jul 6. Epub 2021 Jul 6.

University of Utah Molecular Medicine Program Department of Internal Medicine, University of Utah, Salt Lake City, Utah, USA.

Purpose Of Review: Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus-2. Over the past year, COVID-19 has posed a significant threat to global health. Although the infection is associated with mild symptoms in many patients, a significant proportion of patients develop a prothrombotic state due to a combination of alterations in coagulation and immune cell function. Read More

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Immunoregulatory role of circulating endothelial vWF positive cells in patients after acute myocardial infarction.

J Biol Regul Homeost Agents 2021 May-Jun;35(3):1159-1168

Department of Physiology and Immunology, Faculty of Medicine, University of Rijeka, Rijeka, Croatia.

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Study of serum level and immunohistochemical expression of von Willebrand factor in psoriasis.

J Immunoassay Immunochem 2021 Jul 6:1-13. Epub 2021 Jul 6.

Pathology Department, Menoufia University Faculty of Medicine, Shebin El-Kom, Egypt.

Von Willebrand factor (vWF) is angiogenic, hypercoagulable, and inflammatory marker that increases inflammation and vasculitis and reflects endothelial cells dysfunction. vWF could play a role in psoriasis pathogenesis and prognosis. To assess the serum and immunohistochemical expression of vWF in psoriasis to evaluate its possible role in disease pathogenesis and prognosis. 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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Hemostatic state of children with type 1 diabetes.

Ann Pediatr Endocrinol Metab 2021 Jun 30;26(2):99-104. Epub 2021 Jun 30.

Departamento de Bioquímica Aplicada, Facultad de Bioquímica, Universidad Nacional de Tucumán, Tucumán, Argentina.

Purpose: Hyperglycemia is one of the factors responsible for the molecular alterations that modify hemostasis. The aim of this study was to determine the levels of circulating molecules that have a prothrombotic impact on the child and adolescent population with type 1 diabetes mellitus.

Methods: There were 35 patients with type 1 diabetes mellitus (11. Read More

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