13,365 results match your criteria von Willebrand Disease


Trans women have worse cardiovascular biomarker profiles than cisgender men independent of hormone use and HIV serostatus.

AIDS 2022 Aug 11. Epub 2022 Aug 11.

University of California Los Angeles, Los Angeles, CA, USA.

Background: Feminizing hormonal therapy (FHT) and HIV potentially alter cardiovascular disease (CVD) risk in transgender women (TW).

Methods: TW were enrolled in Los Angeles, CA and Houston, TX and frequency-matched to Multicenter AIDS Cohort Study cisgender men (CM) on age, race, substance use and abacavir use. Biomarkers of CVD risk and inflammation were assessed via ELISA. Read More

View Article and Full-Text PDF

Successful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report.

Rheumatol Ther 2022 Aug 10. Epub 2022 Aug 10.

Department of Pathophysiology and Transplantation, University of Milan, Via Pace, 9, 20122, Milan, Italy.

Acquired von Willebrand syndrome (AVWS) is a rare, non-hereditary bleeding disorder related to heterogeneous medical conditions such as hematological malignancies and cardiovascular and autoimmune diseases. We describe the clinical course of a 62-year-old man with polycythemia vera who experienced post-traumatic knee and leg swelling due to hemarthrosis. He was treated at another center with low molecular weight heparin due to misdiagnosed deep vein thrombosis further exacerbating the ongoing bleeding. Read More

View Article and Full-Text PDF

Purtscher-like retinopathy in a patient with COVID-19-associated coagulopathy.

Arq Bras Oftalmol 2022 Aug 8. Epub 2022 Aug 8.

Department of Medicine (Infectious Diseases), Jacobi Medical Center, Bronx, NY.

The most frequently reported ophthalmic manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is conjunctivitis. We have described a case of Purtscher-like retinopathy in a patient with severe coronavirus disease 2019 (COVID-19)-associated coagulopathy. A young woman with multiple comorbidities was admitted for COVID-19-related acute respiratory distress syndrome. Read More

View Article and Full-Text PDF

Von Willebrand factor (vWF) in patients with heart failure with preserved ejection fraction (HFpEF): A retrospective observational study.

Medicine (Baltimore) 2022 Aug;101(31):e29854

Department of Cardiology, The Fifth Affiliated Hospital of Xinjiang Medical University, Urumqi, People's Republic of China.

Heart failure with preserved ejection fraction (HFpEF) is associated with endothelial damage and inflammation. In addition, von Willebrand factor (vWF) has been discovered as a biomarker of endothelial dysfunction. Therefore, the study aims to investigate the association between vWF level and HFpEF. Read More

View Article and Full-Text PDF

Microsurgical Breast Reconstruction in Patients with Disorders of Hemostasis - Perioperative Risks and Management.

Plast Reconstr Surg 2022 Aug 10. Epub 2022 Aug 10.

Jian Farhadi, M.D., Prof. Plastic Surgery Group; Zurich, Switzerland. University of Basel; Basel, Switzerland.

Background: Surgical and technological advances have resulted in the widespread adoption of microsurgical breast reconstruction. Many comorbidities that potentially might impair vasculature and wound healing are no longer considered contraindications for these procedures. However, some uncertainty still prevails regarding the perioperative management of patients with disorders of hemostasis. Read More

View Article and Full-Text PDF

Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis.

Hematology 2022 Dec;27(1):860-866

Department of Medical Laboratory Science, Faculty of Health Sciences, University of Nigeria Enugu Campus, Nsukka, Nigeria.

Aim: To evaluate the contributions of VWF to the clinical manifestation and severity of SCD.

Design: A systematic review of peer-reviewed articles published in English. The review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Read More

View Article and Full-Text PDF
December 2022

The monomeric C-reactive protein level is associated with the increase in carotid plaque number in patients with subclinical carotid atherosclerosis.

Front Cardiovasc Med 2022 22;9:968267. Epub 2022 Jul 22.

Laboratory of Cell Hemostasis, National Medical Research Centre of Cardiology named after academician E.I. Chazov of the Ministry of Health of the Russian Federation, Moscow, Russia.

The high-sensitivity C-reactive protein (hsCRP) assay measures the level of the pentameric form of CRP in blood. Currently, there are no available assays measuring the level of the monomeric form of CRP (mCRP), produced at sites of local inflammation. We developed an assay measuring the mCRP level in blood plasma with functional beads for flow cytometry. Read More

View Article and Full-Text PDF

A COVID Constellation: A Case of Transverse Myelitis and Acquired von Willebrand Syndrome.

J Investig Med High Impact Case Rep 2022 Jan-Dec;10:23247096221109205

Emory University School of Medicine, Atlanta, GA, USA.

The coronavirus disease 2019 (COVID-19) pandemic revealed a myriad of postinfectious severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) sequelae, many of which remain poorly understood. We describe a rare presentation of a patient developing 2 simultaneous COVID-19 sequelae: transverse myelitis and acquired von Willebrand syndrome (AVWS). There have been numerous published case reports of patients developing transverse myelitis after a diagnosis of COVID-19. Read More

View Article and Full-Text PDF

High Prevalence of Congenital Factor VII (FVII) Deficiency in Adolescent Females with Heavy Menstrual Bleeding and Iron Deficiency Anemia.

J Pediatr Adolesc Gynecol 2022 Jul 30. Epub 2022 Jul 30.

Holtz Children's Hospital - Jackson Memorial Medical Center. Miami, FL; Division of Pediatric Hematology-Oncology, Department of Pediatrics. University of Miami - Miller School of Medicine. Miami, FL; University of Miami - Hemophilia Treatment Center. Mami, FL. Electronic address:

Study Objective: To examine the clinical characteristics and prevalence of congenital bleeding disorders (CBDs), with emphasis on congenital factor VII (FVII) deficiency and other rare bleeding disorders, in adolescent and young adult females referred to a Hemophilia Treatment Center (HTC) for evaluation and management of heavy menstrual bleeding (HMB) and iron deficiency anemia (IDA).

Design: This single-center retrospective study reviewed the clinical characteristics and prevalence of CBDs in post-menarcheal females, younger than 22-years-of-age, referred to an HTC from 2015 to 2021 for evaluation of HMB with or without IDA.

Results: One hundred females, mean age of 15 years (range 9 to 20 years), met initial study criteria with 95 included in the final analysis. Read More

View Article and Full-Text PDF

Cardiac gene activation varies between young and adult cats and in the presence of hypertrophic cardiomyopathy.

Res Vet Sci 2022 Jul 23;152:38-47. Epub 2022 Jul 23.

Department of Clinical Studies, Ontario Veterinary College, University of Guelph, 50 Stone Road E., Guelph, ON N1G 2W1, Canada. Electronic address:

Little is known about the difference of myocardial gene transcription in young and adult cats and how transcription is further modified in cats with hypertrophic cardiomyopathy (HCM) and with left atrial (LA) thrombus formation. We hypothesized that selected factors for coagulation, endothelial activation, inflammation, and remodelling are modified with age and are activated in the hearts of cats with HCM. Left atrial and ventricular (LV) samples from 12 cats with HCM (seven without (HCMwoAT] and five with LA thrombi [HCMwAT]), and six young (YC) and six adult (AC) control cats without cardiac disease were investigated for relative expression of the following genes using quantitative polymerase chain reaction: von Willebrand factor, a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13, platelet activating factor, E- and P-selectin, intercellular and vascular adhesion molecules-1, ß2-integrin, vascular endothelial growth factor, interleukin-6 (IL-6), monocyte chemoattractant protein-1 (MCP-1), heat shock protein-70, and myocyte-specific enhancer factor 2C. Read More

View Article and Full-Text PDF

Chronic intermittent hypoxia promotes early intrahepatic endothelial impairment in rats with non-alcoholic fatty liver disease.

Am J Physiol Gastrointest Liver Physiol 2022 Aug 2. Epub 2022 Aug 2.

Liver Unit, University Hospital of the Canary Islands, Tenerife, Spain.

Non-alcoholic fatty liver disease (NAFLD) is a progressive disease that ranges from simple steatosis to cirrhosis. Obstructive sleep apnea syndrome (OSAS) and chronic intermittent hypoxia (CIH), are implicated in the pathogenesis of NAFLD. However, the overlapping consequences of CIH on liver sinusoidal endothelial function over time in NAFLD are largely unknown. Read More

View Article and Full-Text PDF

Aberrant Expression of ACO1 in Vasculatures Parallels Progression of Idiopathic Pulmonary Fibrosis.

Front Pharmacol 2022 15;13:890380. Epub 2022 Jul 15.

Division of Allergy and Immunology, Department of Internal Medicine, University of South Florida, Tampa, FL, United States.

Idiopathic pulmonary fibrosis (IPF) is characterized by mitochondrial dysfunction. However, details about the non-mitochondrial enzymes that sustain the proliferative nature of IPF are unclear. Aconitases are a family of enzymes that sustain metabolism inside and outside mitochondria. Read More

View Article and Full-Text PDF

LncRNA ANRIL mediates endothelial dysfunction through BDNF downregulation in chronic kidney disease.

Cell Death Dis 2022 Jul 29;13(7):661. Epub 2022 Jul 29.

Department of Nephrology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, 250021, Jinan, Shandong, China.

Endothelial dysfunction is common in patients with chronic kidney disease (CKD), but the mechanism is unknown. In this study, we found that the circulating ANRIL level was increased and correlated with vascular endothelial dysfunction in patients with CKD, also negatively correlated with plasma brain-derived neurotrophic factor (BDNF) concentration. We constructed the ANRIL knockout mice model, and found that ANRIL deficiency reversed the abnormal expression of BDNF, along with endothelial nitric oxide synthase (eNOS), vascular adhesion molecule 1 (VCAM-1) and Von Willebrand factor (vWF). Read More

View Article and Full-Text PDF

Prolonged APTT and autoimmune overt hypothyroidism identified postpartum: a case report.

Eur Thyroid J 2022 Aug 15;11(4). Epub 2022 Jul 15.

Department of Clinical Biochemistry, Aalborg University Hospital, Aalborg, Denmark.

Introduction: Thyroid disorders have been linked to abnormalities in the coagulation system, and a hypocoagulant state has been proposed in hypothyroidism. The assessment of thyroid function is, however, not routinely recommended as part of the assessment for coagulation disorders.

Case Presentation: We present a 32-year-old woman who had no history of thyroid disease and who recently gave birth preterm because of severe preeclampsia and intrauterine growth restriction. Read More

View Article and Full-Text PDF

Noninvasive fetal genotyping of single nucleotide variants and linkage analysis for prenatal diagnosis of monogenic disorders.

Hum Genomics 2022 Jul 27;16(1):28. Epub 2022 Jul 27.

Obstetrics and Gynecology Hospital, Institute of Reproduction and Development, Fudan University, Shanghai, People's Republic of China.

Background: High-cost, time-consuming and complex processes of several current approaches limit the use of noninvasive prenatal diagnosis (NIPD) for monogenic disorders in clinical application. Thus, a more cost-effective and easily implementable approach is required.

Methods: We established a low-cost and convenient test to noninvasively deduce fetal genotypes of the mutation and single nucleotide polymorphisms (SNPs) loci by means of targeted amplification combined with deep sequencing of maternal genomic and plasma DNA. Read More

View Article and Full-Text PDF

Acquired Thrombotic Thrombocytopenic Purpura Following Inactivated COVID-19 Vaccines: Two Case Reports and a Short Literature Review.

Vaccines (Basel) 2022 Jun 24;10(7). Epub 2022 Jun 24.

Medical Intensive Care Unit, Faculty of Medicine of Sousse, University of Sousse, Sousse 4000, Tunisia.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak in December 2019, causing millions of deaths all over the world, and the lack of specific treatment for severe forms of coronavirus disease 2019 (COVID-19) have led to the development of vaccines in record time, increasing the risk of vaccine safety issues. Recently, several cases of thrombotic thrombocytopenic purpura (TTP) have been reported following COVID-19 vaccination. TTP is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia and ischemic end-organ lesions. Read More

View Article and Full-Text PDF

Von Willebrand Factor and ADAMTS-13 Are Associated with the Severity of COVID-19 Disease.

J Clin Med 2022 Jul 11;11(14). Epub 2022 Jul 11.

National Medical Research Center for Obstetrics, Gynecology and Perinatology Named after Academician V.I. Kulakov of Ministry of Healthcare of Russian Federation, 117997 Moscow, Russia.

Coagulopathy in COVID-19 patients is presumably based on systemic hypercoagulation with the inflammatory response. As a result of endothelial dysfunction, tissue factor and von Willebrand factor (vWF) are released into the blood stream, which leads to prothrombinase activation. The vWF/ADAMTS-13 ratio can be used for monitoring the severity of the disease. Read More

View Article and Full-Text PDF

Effect of Low High-Density Lipoprotein Level on Endothelial Activation and Prothrombotic Processes in Coronary Artery Disease-A Pilot Study.

Int J Environ Res Public Health 2022 Jul 15;19(14). Epub 2022 Jul 15.

Department of Medical Biology and Biochemistry, Faculty of Medicine, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 87-100 Toruń, Poland.

High-density lipoproteins (HDL) play an important role in the prevention of atherosclerosis. The aim of the study was to assess the relationship between serum HDL-C concentration and proinflammatory/prothrombic activation in coronary artery disease (CAD) patients. The study group included 27 acute myocardial infarction (AMI) patients and 30 stable angina pectoris (SA) patients. Read More

View Article and Full-Text PDF

A Race against the Clock: A Case Report and Literature Review Concerning the Importance of ADAMTS13 Testing in Diagnosis and Management of Thrombotic Thrombocytopenic Purpura during Pregnancy.

Diagnostics (Basel) 2022 Jun 27;12(7). Epub 2022 Jun 27.

Department of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, Romania.

Thrombocytopenic purpura (TTP) is a rare, potentially fatal pathology characterized by microangiopathic thrombotic syndrome and caused by an acute protease deficiency of von Willebrand factor, ADAMTS13. Moreover, ADAMTS13 deficiency promotes microthrombosis led by the persistence of ultra-large VWF multimers in the blood circulation. According to the few studies involving pregnant participants, the heterogeneity of manifestations has made this pathology difficult to diagnose, with an unexpected occurrence and increased risk of maternal and fetal morbidity and mortality. Read More

View Article and Full-Text PDF

High levels of von Willebrand factor with reduced specific activities in hospitalized patients with or without COVID-19.

J Thromb Thrombolysis 2022 Aug 26;54(2):211-216. Epub 2022 Jul 26.

Center for Translational Research on Inflammatory Diseases (CTRID), Michael E. DeBakey VA Medical Center, Houston, USA.

The COVID-19 pandemic is often accompanied by severe respiratory illness and thrombotic complications. Von Willebrand Factor (VWF) levels are highly elevated in this condition. However, limited data are available on the qualitative activity of VWF in COVID-19. Read More

View Article and Full-Text PDF

Cerebral sinus venous thrombosis in children with inherited bleeding disorders: A case series.

Pediatr Blood Cancer 2022 Jul 26:e29902. Epub 2022 Jul 26.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

In patients with inherited bleeding disorders, thrombus development poses a challenge in balancing the management of thrombosis and bleeding. Pediatric antithrombotic therapy guidelines do not address the treatment of a thrombus in the setting of a bleeding disorder. We present a case series of four children with inherited bleeding disorders presenting with cerebral sinus venous thrombosis and bleeding, in order to summarize the different therapeutic approaches and outcomes of these patients. Read More

View Article and Full-Text PDF

Arterial stiffness and atherosclerosis in systemic lupus erythematosus patients.

Reumatologia 2022 13;60(3):165-172. Epub 2022 Jul 13.

Department of Cardiac and Vascular Diseases, John Paul II Hospital, Jagiellonian University Medical College, Krakow, Poland.

Introduction: Systemic lupus erythematosus (SLE) is characterized by early atherothrombosis. Pulse wave velocity (PWV) is a promising tool for the diagnosis of early vascular remodelling and initial atherosclerotic plaque formation. Our objective was to evaluate PWV and its relationship with coronary atherosclerosis and thrombotic biomarkers in patients with SLE. Read More

View Article and Full-Text PDF

Clinical significance of substantially elevated von Willebrand factor antigen levels in patients with advanced chronic liver disease.

Dig Liver Dis 2022 Jul 21. Epub 2022 Jul 21.

Department of Internal Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria.

Background: Von Willebrand factor antigen (VWF) is a non-invasive marker for clinically significant portal hypertension (HVPG≥10 mmHg) and confers HVPG-independent prognostic information. While quantification of increased VWF-levels is not relevant in the context of von Willebrand disease, highly elevated VWF may be of clinical significance in ACLD. Thus, we have modified our analytical approach to quantify very high VWF-levels (i. Read More

View Article and Full-Text PDF

Acquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome.

Intern Med 2022 Jul 22. Epub 2022 Jul 22.

Department of Hematology, Fukushima Medical University, Japan.

Acquired von Willebrand syndrome (aVWS) develops with various underlying diseases. We herein report an individual with aVWS associated with mucosa-associated lymphoid tissue lymphoma in the lungs complicated by hyperviscosity syndrome, Sjögren's syndrome, and hypothyroidism. This patient developed life-threatening hemorrhaging during a lung biopsy despite transfusion of concentrate of plasma-derived VWF/factor VIII. Read More

View Article and Full-Text PDF

[Association between coagulation function and prognosis in patients with acute pancreatitis].

Nan Fang Yi Ke Da Xue Xue Bao 2022 Jul;42(7):1006-1012

Department of Laboratory Medicine, West China Hospital, Sichuan University, Chengdu 610041, China.

Objective: To explore the correlation of coagulation function with the severity and prognosis of acute pancreatitis (AP) and identify the laboratory markers for early prediction and dynamic monitoring of the prognosis of AP.

Methods: We retrospectively analyzed the clinical data of patients with AP admitted less than 72 h after onset to our hospital from December 1, 2017 to November 30, 2018. The correlation of coagulation function-related markers at admission and their changes during hospitalization with the prognosis of the patients was analyzed. Read More

View Article and Full-Text PDF

Neutrophil extracellular traps promote thrombogenicity in cerebral venous sinus thrombosis.

Cell Biosci 2022 Jul 22;12(1):114. Epub 2022 Jul 22.

Department of Neurosurgery, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, China.

Background: Neutrophil extracellular traps (NETs) contribute to the creation of a coagulation state in various diseases. Currently, it is not clear whether NETs are present in the thrombi and plasma of patients with cerebral venous sinus thrombosis (CVST). This study aimed to investigate the presence of NETs in thrombi and blood samples from CVST patients and the procoagulant activity (PCA) of NETs during the progression of CVST. Read More

View Article and Full-Text PDF

Thromboinflammation: From Atherosclerosis to COVID-19.

Arterioscler Thromb Vasc Biol 2022 Jul 8:101161ATVBAHA122317162. Epub 2022 Jul 8.

Cellular and Molecular Medicine, Boston Children's Hospital/Harvard Medical School, MA (D.D.W.).

The activating interplay of thrombosis and inflammation (thromboinflammation) has been established as a major underlying pathway, driving not only cardiovascular disease but also autoimmune disease and most recently, COVID-19. Throughout the years, innate immune cells have emerged as important modulators of this process. As the most abundant white blood cell in humans, neutrophils are well-positioned to propel thromboinflammation. Read More

View Article and Full-Text PDF

Sixth Åland Island Conference on von Willebrand disease.

Haemophilia 2022 Jul;28 Suppl 5:3-15

Bleeding & Clotting Disorders Institute, Peoria, Illinois, USA.

Introduction: The sixth Åland Islands Conference on von Willebrand disease (VWD) on the Åland Islands, Finland, was held from 20 to 22 September 2018.

Aim: The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD.

Results And Discussion: The topics covered both clinical aspects of disease management, and biochemical and laboratory insights into the disease. Read More

View Article and Full-Text PDF

Towards 50 years of platelet function analyser (PFA) testing.

Clin Chem Lab Med 2022 Jul 21. Epub 2022 Jul 21.

Section of Clinical Biochemistry and School of Medicine, University of Verona, Verona, Italy.

The platelet function analyser (PFA) is a prevalent platelet function screening instrument, and comes in two models-the original PFA-100 and the contemporary PFA-200. The instruments have 'identical' output, being a 'closure time' (CT). Moreover, normal reference ranges provided by the manufacturer, for the specific test cartridges, are the same for both models. Read More

View Article and Full-Text PDF