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Von Willebrand Factor

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https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#1
REVIEW
Marcel Levi, Marie Scully, Mervyn Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor is most prominent. The multimeric size of von Willebrand factor is an important determinant of a more intense platelet-vessel wall interaction. and is regulated by the von Willebrand factor cleaving protease, ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultra-large von Willebrand factor multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura (TTP), a thrombotic microangiopathy characterized by thrombocytopenia, non-immune haemolysis, and organ dysfunction...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29337406/validation-of-flow-cytometric-analysis-of-platelet-function-in-patients-with-a-suspected-platelet-function-defect
#2
Ivar van Asten, Roger E G Schutgens, Marije Baaij, Judith Zandstra, Mark Roest, Gerard Pasterkamp, Albert Huisman, Suzanne J A Korporaal, Rolf T Urbanus
BACKGROUND: Light transmission aggregometry (LTA) is the most commonly used test for the diagnosis of platelet function disorders (PFDs), but has moderate sensitivity for mild PFDs. Flow cytometry has been recommended for additional diagnostics of PFDs but is not yet standardized as a diagnostic test. We developed a standardized protocol for flow cytometric analysis of platelet function that measures fibrinogen binding and P-selectin expression as platelet activation markers in response to agonist stimulation...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29336529/high-prevalence-of-reduced-thrombin-generation-and-or-decreased-platelet-response-in-women-with-unexplained-heavy-menstrual-bleeding
#3
H P Eising, M Roest, P G de Groot, D Huskens, J Konings, R T Urbanus, B de Laat, J A Remijn
INTRODUCTION: Heavy menstrual bleeding (HMB) is a condition that affects 20%-30% of women of reproductive age. HMB has a multifactorial pathophysiology, which is incompletely understood. HMB symptoms are very common in patients with established haemostasis defects, likewise, women with heavy menstrual bleeding have a higher prevalence of impaired Von Willebrand factor (VWF) levels and function, thrombocytopenia, impaired platelet function and impaired coagulation. The aim of this study was to quantify the prevalence of impaired platelet function, impaired coagulation and reduced VWF activity in patients with HMB...
January 16, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29334682/body-mass-index-and-plasma-p-selectin-before-coronary-stenting-predict-high-residual-platelet-reactivity-at-6-months-on-dual-antiplatelet-therapy
#4
Elena Z Golukhova, Marina V Grigoryan, Mariya N Ryabinina, Naida I Bulaeva, Victor L Serebruany
BACKGROUND: High residual platelet reactivity (HRPR) during dual antiplatelet therapy (DAPT) may impact clinical outcomes following percutaneous coronary interventions (PCI). However, whether any biomarkers assessed before PCI at DAPT loading may predict delayed maintenance HRPR is not clear. OBJECTIVE: The aim of this study was to determine whether conventional clinical or laboratory indices at loading before stenting may predict HRPR at 6 months of maintenance DAPT...
January 16, 2018: Cardiology
https://www.readbyqxmd.com/read/29334169/impact-of-baseline-clinical-and-laboratory-features-on-the-risk-of-thrombosis-in-children-with-acute-lymphoblastic-leukemia-a-prospective-evaluation
#5
Uma H Athale, T Mizrahi, C Laverdière, T Nayiager, Y-L Delva, G Foster, L Thabane, M David, J-M Leclerc, A K C Chan
BACKGROUND: Children with acute lymphoblastic leukemia (ALL) have increased risk of thromboembolism (TE). However, the predictors of ALL-associated TE are as yet uncertain. OBJECTIVE: This exploratory, prospective cohort study evaluated the effects of clinical (age, gender, ALL risk group) and laboratory variables (hematological parameters, ABO blood group, inherited and acquired prothrombotic defects [PDs]) at diagnosis on the development of symptomatic TE (sTE) in children (aged 1 to ≤18) treated on the Dana-Farber Cancer Institute ALL 05-001 study...
January 15, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29331958/platelet-secretion-defects-and-acquired-von-willebrand-syndrome-in-patients-with-ventricular-assist-devices
#6
Ulrich Geisen, Kerstin Brehm, Georg Trummer, Michael Berchtold-Herz, Claudia Heilmann, Friedhelm Beyersdorf, Johannes Schelling, Axel Schlagenhauf, Barbara Zieger
BACKGROUND: The number of implanted ventricular assist devices (VADs) has increased significantly recently. Bleeding, the most frequent complication, cannot be solely attributed to anticoagulation therapy. Acquired von Willebrand syndrome (AVWS) caused by increased shear stress is frequent in VAD patients and can increase the bleeding risk. The HeartMate III (HM III) is a novel left VAD featuring potential improvements over the HeartMate II. METHODS AND RESULTS: In this study, we investigated the prevalence and onset of AVWS in 198 VAD patients...
January 13, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29328041/von-willebrand-factor-immunohistochemical-staining-quantitative-optical-density-parameters-in-the-endothelium-and-fibrinoid-of-the-placenta-during-secundines-inflammation-and-concomitant-iron-deficiency-anemia-in-gravidas
#7
V Ilika, I Davydenko
The aim of the research was to set the optical density quantitative parameters of the von Willebrand factor immunohistochemical staining (vWF) in the endothelium and fibrinoid of the placenta during the secundines inflammation concomitant with iron deficiency anemia in gravidas. The total number of 198 placentas was studied. The immunohistochemical technique was performed using the visualization of the primary antibodies to vWF with a diaminobenzidine dye polymer system. The optical density of the histochemical staining was measured by means of computer microdensitometry after the digital copies of the images had been obtained...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29326120/macrophage-scavenger-receptor-sr-ai-contributes-to-the-clearance-of-von-willebrand-factor
#8
Nikolett Wohner, Vincent Muczynski, Amel Mohamadi, Paulette Legendre, Gabriel Aymé, Olivier D Christophe, Peter J Lenting, Cécile V Denis, Caterina Casari
Previously, we identified LDL-receptor related protein-1 on macrophages to mediate shear stress-dependent clearance of von Willebrand factor. In control experiments, however, we observed that von Willebrand factor also binds to macrophages independently of this receptor under static conditions, suggesting the existence of additional clearance-receptors. In search for such receptors, we focused on the macrophage-specific scavenger-receptor SR-AI. Von Willebrand factor displays efficient binding to SR-AI (half-max binding 14+/-5 nM)...
January 11, 2018: Haematologica
https://www.readbyqxmd.com/read/29323038/electroacupuncture-improves-neurovascular-unit-reconstruction-by-promoting-collateral-circulation-and-angiogenesis
#9
Lei Shi, Hong-Mei Cao, Ying Li, Shi-Xin Xu, Yan Zhang, Yang Zhang, Zhe-Feng Jin
Acupuncture at Shuigou (GV26) shows good clinical efficacy for treating stroke, but its mechanism remains poorly understood. In this study, a cerebral infarction model of ischemia/reperfusion injury received electroacupuncture at GV26 (15 Hz and 1 mA, continuous wave [biphasic pulses], for 5 minutes). Electroacupuncture effectively promoted regional cerebral blood flow on the infarct and non-infarct sides, increased infarct lesions, lectin, and number of blood vessels, upregulated von Willebrand factor and cell proliferation marker Ki67 expression, and diminished neurological severity score...
December 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/29321442/acquired-von-willebrand-syndrome-due-to-aortic-valve-stenosis-in-a-case-with-antiphospholipid-antibody
#10
Hiroaki Tanaka, Yurie Nagai, Chihiro Kuwabara, Ryo Shimizu, Akihide Umeki, Tetsufumi Yamamoto
Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29319374/sensitive-and-specific-assessment-of-recombinant-von-willebrand-factor-in-platelet-function-analyzer
#11
Isabell Pekrul, Thorsten Kragh, Peter L Turecek, Aaron R Novack, Helmut W Ott, Michael Spannagl
BACKGROUND: Recombinant von Willebrand factor (rVWF), which was licensed in the United States in 2015, has the multimeric distribution of freshly secreted VWF with ultralarge (UL) and high molecular weight multimers (HMWM) from endothelial cells and megakaryocytes since it has never been exposed to ADAMTS13 or any other proteolytic enzyme. Measurement of closure time (CT) using the platelet function analyzer-200 (PFA-200) is highly sensitive to the presence of UL VWF multimers in added VWF concentrates...
January 10, 2018: Platelets
https://www.readbyqxmd.com/read/29317405/coagulation-and-fibrinolysis-in-hyperparathyroidism-secondary-to-vitamin-d-deficiency
#12
Laura P B Elbers, Marije Wijnberge, Joost C M Meijers, Dennis C W Poland, Dees P M Brandjes, Eric Fliers, Victor E A Gerdes
INTRODUCTION: Abnormal coagulation tests have been observed in patients with primary hyperparathyroidism (HPT) suggesting a prothrombotic effect of parathyroid hormone (PTH). Vitamin D deficiency (VIDD) is the most frequent cause of secondary HPT. Aim of our study was to investigate the influence of HPT secondary to moderate-severe VIDD and vitamin D replacement on the coagulation and fibrinolysis system. SUBJECTS AND METHODS: Prospective cohort study of patients with vitamin D<25 nmol/l with and without HPT, and a control group of patients on vitamin D suppletion...
January 9, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29315844/local-and-systemic-coagulation-marker-response-to-musculocutaneous-flap-ischemia-reperfusion-injury-and-remote-ischemic-conditioning-an-experimental-study-in-a-porcine-model
#13
Andreas Engel Krag, Christine Lodberg Hvas, Birgitte Jul Kiil, Gete Toft Eschen, Tine Engberg Damsgaard, Anne-Mette Hvas
BACKGROUND: Remote ischemic conditioning (RIC) administered by non-lethal periods of extremity ischemia and reperfusion attenuates ischemia-reperfusion injury. We aimed to investigate the local and systemic coagulation marker response to flap ischemia-reperfusion injury, and the effects of RIC on coagulation markers following flap ischemia-reperfusion injury. METHODS: A musculocutaneous latissimus dorsi flap was subjected to 4 h of ischemia followed by 7 h of reperfusion in 16 female Danish Landrace pigs (39 kg)...
January 8, 2018: Microsurgery
https://www.readbyqxmd.com/read/29315665/effect-of-remote-ischemic-preconditioning-on-left-atrial-remodeling-and-prothrombotic-response-after-radiofrequency-catheter-ablation-for-atrial-fibrillation
#14
Ruijuan Han, Xiaoqing Liu, Meili Zheng, RuiPing Zhao, XiaoYan Liu, Xiandong Yin, Xingpeng Liu, Ying Tian, Liang Shi, Kai Sun, Xinchun Yang
BACKGROUND: Radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF) is known to induce left atrial remodeling and prothrombotic response. AIMS: This study aimed to evaluate the effect of remote ischemic preconditioning (RIPC) on left atrial remodeling and prothrombotic response induced by radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF). METHODS: Forty-four patients with drug-refractory paroxysmal AF undergoing RFCA were randomized into RIPC (4 short episodes of forearm ischemia) and control groups before the procedure...
January 5, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29315472/early-prognostic-factors-in-septic-shock-cancer-patients-a-prospective-study-with-a-proteomic-approach
#15
D Mokart, C Saillard, C Zemmour, M Bisbal, A Sannini, L Chow-Chine, J-P Brun, M Faucher, J-M Boher, Y Toiron, C Chabannon, J-P Borg, A Gonçalves, L Camoin
BACKGROUND: Organ failures are the main prognostic factors in septic shock. The aim was to assess classical clinico-biological parameters evaluating organ dysfunctions at intensive care unit admission, combined with proteomics, on day-30 mortality in critically ill onco-hematology patients admitted to the intensive care unit for septic shock. METHODS: This was a prospective monocenter cohort study. Clinico-biological parameters were collected at admission. Plasma proteomics analyses were performed, including protein profiling using isobaric Tag for Relative and Absolute Quantification (iTRAQ) and subsequent validation by ELISA...
January 7, 2018: Acta Anaesthesiologica Scandinavica
https://www.readbyqxmd.com/read/29314439/low-incidence-of-factor-viii-inhibitors-in-previously-untreated-patients-with-severe-haemophilia-a-treated-with-octanate%C3%A2-final-report-from-a-prospective-study
#16
A Klukowska, V Komrska, V Vdovin, A Pavlova, M Jansen, S Lowndes, L Belyanskaya, O Walter, P Laguna
INTRODUCTION: Octanate® is a human, plasma-derived, von Willebrand factor-stabilized coagulation factor VIII (FVIII) concentrate with demonstrated haemostatic efficacy in previously treated patients with haemophilia A. AIM: This prospective, open-label study aimed to assess the immunogenicity of octanate® in previously untreated patients (PUPs). METHODS: The study monitored development of FVIII inhibitors in 51 PUPs. Tolerability, viral safety, FVIII recovery and efficacy of octanate® for the prevention and treatment of bleeds and in surgical procedures were also assessed...
January 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29313798/current-challenges-in-the-diagnosis-and-management-of-patients-with-inherited-von-willebrand-s-disease-in-italy-an-expert-meeting-report-on-the-diagnosis-and-surgical-and-secondary-long-term-prophylaxis
#17
Piercarla Schinco, Giancarlo Castaman, Antonio Coppola, Dorina Cultrera, Cosimo Ettorre, Anna C Giuffrida, Emanuela Marchesini, Renato Marino, Marta Milan, Claudio Molinari, Simona M Siboni, Ezio Zanon, Augusto B Federici
Recent advances in the care of von Willebrand 's disease (vWD) have allowed the majority of patients to be managed adequately. Even in the more severe forms, it is now possible to control recurrent bleeding through secondary long-term prophylaxis with von Willebrand factor-containing concentrates. Moreover, in the setting of surgical prophylaxis, the combination of interdisciplinary management and close patient monitoring yields a positive outcome in nearly all cases, although safety concerns remain. In clinical practice, the effectiveness of therapy is hindered by the difficulties in making a rapid, yet accurate diagnosis, in identifying the subgroup of bleeders who may benefit most from a specific strategy, and in selecting the optimal product and regimen...
May 26, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29311493/cellular-atypia-is-negatively-correlated-with-immunohistochemical-reactivity-of-cd31-and-vwf-expression-levels-in-canine-hemangiosarcoma
#18
Aprilia Maharani, Keisuke Aoshima, Shinichi Onishi, Kevin Christian Montecillo Gulay, Atsushi Kobayashi, Takashi Kimura
Canine hemangiosarcoma (HSA) is one of the most common mesenchymal tumors in dogs. Its high metastatic and growth rates are usually associated with poor prognosis. Neoplastic cells of HSA can show various levels of cellular atypia in the same mass and may consist of various populations at different differentiated stages. Up to present, however, there is no report analyzing their differentiation states by comparing cellular atypia with differentiation-related protein expressions. To evaluate whether cellular atypia can be used as a differentiation marker in HSA, we analyzed correlation between cellular atypia and intensities of CD31 and von Willebrand Factor (vWF) staining in HSA cases...
January 1, 2018: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/29310791/matrilins
#19
Mats Paulsson, Raimund Wagener
Marilins mediate interactions between macromolecular components of the extracellular matrix, e.g., collagens and proteoglycans. They are composed of von Willebrand factor type A and epidermal growth factor-like domains and the subunits oligomerize via coiled-coil domains. Matrilin-1 and -3 are abundant in hyaline cartilage, whereas matrilin-2 and -4 are widespread but less abundant. Mutations in matrilin genes have been linked to chondrodysplasias and osteoarthritis and recently characterization of matrilin-deficient mice revealed novel functions in mechanotransduction, regeneration, or inflammation...
2018: Methods in Cell Biology
https://www.readbyqxmd.com/read/29306249/biomarker-and-shear-stress-in-secondary-pediatric-pulmonary-hypertension
#20
Ayhan Pektaş, Rana Olguntürk, Serdar Kula, Erman Çilsal, Ayşe Deniz Oğuz, Fatma Sedef Tunaoğlu
Background/aim: Endothelial dysfunction, tissue damage, inflammation, and microthrombosis are involved in the pathogenesis of pulmonary hypertension (PH), which may be present as a complication of congenital heart diseases. This study aims to identify how indicators of endothelial dysfunction (shear stress), tissue damage (brain natriuretic peptide and troponin T), inflammation (C-reactive protein (CRP)), and microthrombosis (D-dimer and von Willebrand factor) are altered in children with congenital left-to-right shunting...
December 19, 2017: Turkish Journal of Medical Sciences
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