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Von Willebrand Factor

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https://www.readbyqxmd.com/read/27903864/oral-thrombin-inhibitor-aggravates-platelet-adhesion-and-aggregation-during-arterial-thrombosis
#1
Tobias Petzold, Manuela Thienel, Ildiko Konrad, Irene Schubert, Ron Regenauer, Boj Hoppe, Michael Lorenz, Annekathrin Eckart, Sue Chandraratne, Carsten Lennerz, Christof Kolb, Daniel Braun, Janina Jamasbi, Richard Brandl, Siegmund Braun, Wolfgang Siess, Christian Schulz, Steffen Massberg
In patients with atrial fibrillation, oral anticoagulation with oral thrombin inhibitors (OTIs), in contrast to vitamin K antagonists (VKAs), associates with a modest increase in acute coronary syndromes (ACSs). Whether this observation is causatively linked to OTI treatment and, if so, whether OTI action is the result of a lower antithrombotic efficacy of OTI compared to VKA or reflects a yet undefined prothrombotic activity of OTI remain unclear. We analyzed platelet function in patients receiving OTI or dose-adapted VKA under static and flow conditions...
November 30, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/27889551/endothelial-dysfunction-and-cardiovascular-risk-factors-in-childhood-acute-lymphoblastic-leukemia-survivors
#2
Paola Giordano, Paola Muggeo, Maurizio Delvecchio, Santa Carbonara, Alberto Romano, Maria Altomare, Gabriella Ricci, Federica Valente, Annapaola Zito, Pietro Scicchitano, Luciano Cavallo, Marco Matteo Ciccone, Nicola Santoro, Maria Felicia Faienza
BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) survivors have increased risk of obesity, metabolic alterations and cardiovascular disease (CVD). Vascular endothelial function has been studied in adult cancers. Limited data exist regarding CVD risk factors among childhood ALL survivors. We aimed to assess endothelial function, metabolic and cardiovascular risk factors in young survivors of childhood ALL. METHODS: Auxological parameters, blood pressure, glucose, lipid profile, hemostatic markers (total adiponectin and high-molecular-weight subfraction, endothelin-1, von Willebrand factor antigen, thrombin-antithrombin complex, D-dimers, fibrinogen), high sensitive C-reactive protein and ultrasound parameters of endothelial function (flow-mediated dilation-FMD, common carotid intima-media thickness-C-IMT, and antero-posterior diameter of infra-renal abdominal aorta-APAO) were assessed in 52 ALL survivors and 34 sex and age-matched controls...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27889498/bloc-2-subunit-hps6-deficiency-affects-the-tubulation-and-secretion-of-von-willebrand-factor-from-mouse-endothelial-cells
#3
Jing Ma, Zhe Zhang, Lin Yang, Janos Kriston-Vizi, Daniel F Cutler, Wei Li
Hermansky-Pudlak syndrome (HPS) is a recessive disorder with bleeding diathesis, which has been linked to platelet granule defects. Both platelet granules and endothelial Weibel-Palade bodies (WPBs) are members of lysosome-related organelles (LROs) whose formation is regulated by HPS protein associated complexes such as BLOC (biogenesis of lysosome organelles complex) -1, -2, -3, AP-3 (adaptor protein complex-3) and HOPS (homotypic fusion and protein sorting complex). Von Willebrand factor (VWF) is critical to hemostasis, which is stored in a highly-multimerized form as tubules in the WPBs...
October 21, 2016: Journal of Genetics and Genomics, Yi Chuan Xue Bao
https://www.readbyqxmd.com/read/27889474/the-von-willebrand-factor-a1-collagen-iii-interaction-is-independent-of-conformation-and-type-2-von-willebrand-disease-phenotype
#4
Venkata R Machha, Alexander Tischer, Laurie Moon-Tasson, Matthew Auton
The blood von Willebrand factor (VWF) mediates platelet adhesion to injured vessels by sequestering platelets from blood flow and depositing them to collagen and other exposed subendothelial matrix proteins. This process of capturing platelets to facilitate formation of platelet plugs occurs through transient interactions with platelet glycoprotein Ibα via the VWF A1 domain which also binds collagen. Using a conformationally diverse collection of natively folded and mutation-induced misfolded von Willebrand Disease (VWD) variants, we test a recently proposed affinity up-regulation hypothesis which states that collagen binding changes the conformation of the A1 domain to a high affinity GPIbα binding competent state...
November 23, 2016: Journal of Molecular Biology
https://www.readbyqxmd.com/read/27885890/use-of-a-thrombopoietin-receptor-agonist-in-von-willebrand-disease-type-2b-p-v1316m-with-severe-thrombocytopenia-and-intracranial-hemorrhage
#5
O Espitia, C Ternisien, C Agard, P Boisseau, C V Denis, M Fouassier
We present here a 63-year old woman with a long history of immune thrombocytopenia. She was hospitalized for a traumatic intracranial hemorrhage with thrombocytopenia. Following inefficient treatment of four platelet transfusions, immunoglobulins, and corticosteroids, we initiated treatment with a thrombopoietin (TPO) receptor agonist (eltrombopag 25 mg/d) with a good efficacy. Her mother and sister also had chronic thrombocytopenia. Clinical history, hemostasis results, and gene analysis revealed von Willebrand disease (VWD) type 2B with the mutation (c...
November 25, 2016: Platelets
https://www.readbyqxmd.com/read/27882137/von-willebrand-factor-and-coagulation-factor-viii-in-moyamoya-disease-associated-with-graves-disease-a-case-report
#6
Shou-Chen Ren, Bao-Qin Gao, Wei-Li Yang, Wei-Xin Feng, Jian Xu, Shao-Wu Li, Yong-Jun Wang
The present study reported the case of a Chinese boy who was diagnosed with Moyamoya disease (MMD) associated with Graves' disease (GD). An overactivation of von Willebrand factor (vWF) and coagulation factor VIII (FVIII) was identified in the plasma of the patient. Thiamazole and metoprolol treatment was thus administrated. After 2 months of treatment, the patient's thyroid function returned to normal and the neurological symptoms improved gradually. At the same time, the activities of vWF and FVIII were depressed...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27879591/comparison-of-endothelial-differentiation-capacities-of-human-and-rat-adipose-derived-stem-cells
#7
Hakan Orbay, Kamaljit Devi, Priscilla A Williams, Tima Dehghani, Eduardo A Silva, David E Sahar
BACKGROUND: The authors compared the endothelial differentiation capacities of human and rat adipose-derived stem cells to determine whether human adipose-derived stem cells can be a source of endothelial cells clinically. METHODS: Human and rat adipose-derived stem cells were harvested and characterized with flow cytometry and trilineage differentiation. Cells from passages III through V were fed with endothelial cell differentiation medium for up to 3 weeks. Cells were harvested after 1, 2, and 3 weeks, and endothelial differentiation was evaluated with quantitative reverse-transcriptase polymerase chain reaction, flow cytometry, and angiogenic sprouting assays...
December 2016: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27879275/von-willebrand-factor-and-adamts13-too-much-or-too-little-of-a-good-thing
#8
EDITORIAL
Brian T Emmer, David Ginsburg, Karl C Desch
No abstract text is available yet for this article.
December 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/27878207/plasma-derived-versus-recombinant-factor-concentrates-in-pups-a-never-ending-debate
#9
Erik Berntorp
Inhibitor development in haemophilia is a serious complication to treatment with factor concentrates. Since the advent of more pure products, especially developed using recombinant DNA technology, some studies have shown an increased incidence of inhibitors in previously untreated patients (PUPs) receiving recombinant products whereas plasma-derived concentrates sometimes have been claimed to have a protective role, probably due to the content of von Willebrand factor (VWF). In fact, experiments indicate that the VWF may block uptake of factor VIII into macrophages for further processing to the immune system...
November 23, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27876311/circulating-biomarkers-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-patients
#10
Francesca Pescini, Ida Donnini, Francesca Cesari, Serena Nannucci, Raffaella Valenti, Valentina Rinnoci, Anna Poggesi, Anna Maria Gori, Betti Giusti, Angela Rogolino, Alessandra Carluccio, Silvia Bianchi, Maria Teresa Dotti, Antonio Federico, Maurizio Balestrino, Enrico Adriano, Rosanna Abbate, Domenico Inzitari, Leonardo Pantoni
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral microangiopathy presenting with variable features, including migraine, psychiatric disorders, stroke, and cognitive decline and variable disability. On neuroimaging, CADASIL is characterized by leukoencephalopathy, multiple lacunar infarcts, and microbleeds. Previous studies suggest a possible role of endothelial impairment in the pathogenesis of the disease...
November 18, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27874231/stability-of-domain-4-of-the-anthrax-toxin-protective-antigen-and-the-effect-of-the-vwa-domain-of-cmg2-on-stability
#11
Sireesha Mamillapalli, Masaru Miyagi, James G Bann
The major immunogenic component of the current anthrax vaccine, anthrax vaccine adsorbed (AVA) is protective antigen (PA). We have shown recently that the thermodynamic stability of PA can be significantly improved by binding to the Von-Willebrand factor A (VWA) domain of capillary morphogenesis protein 2 (CMG2), and improvements in thermodynamic stability may improve storage and long-term stability of PA for use as a vaccine. In order to understand the origin of this increase in stability, we have isolated the receptor binding domain of PA, domain 4 (D4), and have studied the effect of the addition of CMG2 on thermodynamic stability...
November 22, 2016: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/27872768/hip-replacement-surgery-in-14-year-old-girl-with-factor-v-deficiency-haemostatic-treatment-and-thromboprophylaxis
#12
María Eva Mingot-Castellano, Josefina Pérez-Núñez, Lourdes Baeza-Montañez
Factor V (FV) is a pivotal coagulation factor present in plasma and platelets. It plays an essential role in secondary haemostasis acting as a cofactor in the prothrombinase complex, catalysing the conversion of prothrombin to thrombin. There is little evidence on the management of mayor orthopaedic surgery in paediatric or adolescents subjects with this coagulopathy and almost no information about thromboprophylaxis in these situations. We report a case of a hip replacement in a 14-year-old girl with moderate FV deficiency (0...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27872729/mild-bleeders-diagnosis-is-elusive-in-large-number-of-patients
#13
Mrinalini Kotru, Deepti Mutereja, Abhishek Purohit, Seema Tyagi, Manoranjan Mahapatra, Renu Saxena, Hara Prasad Pati
BACKGROUND: Bleeding is a common clinical presentation. Even patients with mild bleeding disorders are extensively investigated for ascertaining the cause. The present study was conducted in order to evaluate the extent of the possibility of diagnosis in mild bleeding disorders. MATERIAL AND METHODS: This was a prospective study of patients referred for work up of mild bleeding for a period of 13 months. A complete blood count, peripheral smear examination, Prothrombin time, Partial Thromboplastin time and Thrombin Time, Platelet Aggregometry test, tests for von Willebrand's disease and Platelet Factor 3 availability were measured...
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27871277/pulmonary-endothelial-activation-caused-by-extracellular-histones-contributes-to-neutrophil-activation-in-acute-respiratory-distress-syndrome
#14
Yanlin Zhang, Li Guan, Jie Yu, Zanmei Zhao, Lijun Mao, Shuqiang Li, Jinyuan Zhao
BACKGROUND: During the acute respiratory distress syndrome (ARDS), neutrophils play a central role in the pathogenesis, and their activation requires interaction with the endothelium. Extracellular histones have been recognized as pivotal inflammatory mediators. This study was to investigate the role of pulmonary endothelial activation during the extracellular histone-induced inflammatory response in ARDS. METHODS: ARDS was induced in male C57BL/6 mice by intravenous injection with lipopolysaccharide (LPS) or exogenous histones...
November 21, 2016: Respiratory Research
https://www.readbyqxmd.com/read/27868463/postural-changes-in-different-stages-of-schizophrenia-is-associated-with-inflammation-and-pain-a-cross-sectional-observational-study
#15
Viviane Batista Cristiano, Michele Fonseca Vieira Szortyka, Maria Inês Lobato, Keila Maria Ceresér, Paulo Belmonte-de-Abreu
OBJECTIVES: To assess the relationship between posture and inflammatory response markers (C-reactive protein [CRP] and von Willebrand factor [vWF]) in schizophrenics. METHODS: Forty patients with stable schizophrenia were divided into early-stage (<10 years since first episode, n = 15) and late-stage (≥10 years since first episode, n = 25) groups. Both groups were compared to controls (n = 26). All participants underwent postural assessment by biophotogrammetry...
November 21, 2016: International Journal of Psychiatry in Clinical Practice
https://www.readbyqxmd.com/read/27867621/von-willebrand-factor-paravalvular-leak-and-a-new-vista-for-tavr
#16
EDITORIAL
Neal S Kleiman, Michael J Reardon
No abstract text is available yet for this article.
October 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/27865966/febuxostat-attenuates-paroxysmal-atrial-fibrillation-induced-regional-endothelial-dysfunction
#17
YanGuang Li, FuKun Chen, Long Deng, Kun Lin, Xiangmin Shi, Shan Zhaoliang, YuTang Wang
BACKGROUND: Paroxysmal atrial fibrillation (PAF) can increase thrombogenesis risk, especially in the left atrium (LA). The exact mechanism is still unclear. OBJECTIVE: We assessed the effects of PAF on endothelial function, and investigated if febuxostat (FX) can attenuate endothelial dysfunction by inhibition of xanthine oxidase (XO). MATERIALS AND METHODS: Eighteen male New Zealand white rabbits were divided randomly into sham-operated (S), PAF (P) or FX+pacing (FP) groups...
November 11, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27865793/multiplexed-mass-spectrometry-monitoring-of-biomarker-candidates-for-osteoarthritis
#18
Patricia Fernández-Puente, Valentina Calamia, Lucía González-Rodríguez, Lucía Lourido, María Camacho-Encina, Natividad Oreiro, Cristina Ruiz-Romero, Francisco J Blanco
: The methods currently available for the diagnosis and monitoring of osteoarthritis (OA) are very limited and lack sensitivity. Being the most prevalent rheumatic disease, one of the most disabling pathologies worldwide and currently untreatable, there is a considerable interest pointed in the verification of specific biological markers for improving its diagnosis and disease progression studies. Considering the remarkable development of targeted proteomics methodologies in the frame of the Human Proteome Project, the aim of this work was to develop and apply a MRM-based method for the multiplexed analysis of a panel of 6 biomarker candidates for OA encoded by the Chromosome 16, and another 8 proteins identified in previous shotgun studies as related with this pathology, in specimens derived from the human joint and serum...
November 16, 2016: Journal of Proteomics
https://www.readbyqxmd.com/read/27865118/dienogest-reduces-proliferation-ngf-expression-and-nerve-fiber-density-in-human-adenomyosis
#19
Arisa Takeuchi, Kaori Koga, Mariko Miyashita, Tomoko Makabe, Fusako Sue, Miyuki Harada, Tetsuya Hirata, Yasushi Hirota, Tomoyuki Fujii, Yutaka Osuga
OBJECTIVES: To evaluate the in vivo effect of dienogest on proliferation, apoptosis, aromatase expression, vascular density, nerve growth factor (NGF) expression and nerve fiber density in human adenomyosis tissue. STUDY DESIGN: Twelve women who underwent hysterectomy for adenomyosis were enrolled. Six patients received dienogest treatment prior to hysterectomy (dienogest group), and age-matched six patients who had not received any hormonal treatment for ≥3 months before surgery (control group)...
November 8, 2016: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/27863189/hemophilia-in-sports-a-case-report-and-prophylactic-protocol
#20
Mark Maffet, Jimmy Roton
OBJECTIVE:   To describe a successful prophylactic protocol for managing an athlete with hemophilia playing at a high level of contact sports. BACKGROUND:   Published data show that team physicians are not comfortable either treating athletes with bleeding disorders or allowing them to participate in contact sports. Much of the literature historically has recommended against allowing athletes with bleeding disorders to play sports at all and certainly against playing contact sports...
November 18, 2016: Journal of Athletic Training
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