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https://www.readbyqxmd.com/read/28092925/risk-based-management-of-dental-procedures-in-patients-with-inherited-bleeding-disorders-development-of-a-dental-bleeding-risk-assessment-and-treatment-tool-debratt
#1
L Rasaratnam, P Chowdary, D Pollard, B Subel, C Harrington, U R Darbar
INTRODUCTION: Successful outcomes in dental management for patients with inherited bleeding disorders require close collaboration between haematology teams and dentists. AIM: To review outcomes of an interdisciplinary pathway for dental procedures by assessing adequacy and appropriateness of haemostatic management. METHODS: Two hundred dental procedures in 30 patients with inherited bleeding disorders were included. A Dental Bleeding Risk Assessment and Treatment Tool (DeBRATT) was developed to identify four categories of bleeding risk (no risk, low, moderate and high risk of bleeding) in relation to the severity of the bleeding disorder and the invasiveness of dental procedure...
January 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28091443/molecular-characterization-of-exon-28-of-von-willebrand-s-factor-gene-in-nigerian-population
#2
E D Ezigbo, E O Ukaejiofo, T U Nwagha
BACKGROUND: Polymorphisms in von Willebrand factor (VWF) gene are an important contributor to the expression of VWF gene and differences in ethnic distribution of these single nucleotide polymorphisms (SNPs) exists. AIMS: Our objective was to molecularly characterize the exon 28 of the VWF gene in the three major ethnic groups of Nigeria. SUBJECTS AND METHODS: We recruited 90 subjects, 45 had a history of bleeding. Questions included those used in the Zimmerman Program for the Molecular and Clinical Biology of von Willebrand disease (VWD), and the bleeding scores were calculated using the Molecular and Clinical Markers for the Diagnosis and Management of type 1 VWD scoring system...
February 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28090741/conformational-quiescence-of-adamts13-prevents-proteolytic-promiscuity-reply
#3
K South, M O Freitas, D A Lane
We thank Drs Cao and Long Zheng for their interest in our paper recently published in JTH [1] in which we reported our findings that C-terminally truncated variants of ADAMTS13 directly cleave the Aα chain of fibrinogen. We postulated that conformationally activated ADAMTS13 [2, 3]may be able to cleave substrate(s) in addition to its usual substrate, the A2 domain of von Willebrand factor (VWF). In their letter, Cao and Long Zheng report that rather than cleaving fibrinogen directly, ADAMTS13 variants act indirectly, activating plasminogen, a common contaminant in commercial fibrinogen preparations...
January 16, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28090594/effects-of-tranexamic-acid-on-platelet-function-and-thrombin-generation-etaplat-woman-trial-sub-study
#4
Kastriot Dallaku, Haleema Shakur, Ian Roberts, Phil Edwards, Danielle Beaumont, Maria Delius, Braun Siegmund, Orion Gliozheni, Ilir Tasha, Saimir Cenameri, Ulrich Mansmann
Background. Postpartum haemorrhage (PPH) is a leading cause of maternal death. Tranexamic acid (TXA) has the potential to reduce bleeding and a large randomized placebo controlled trial of its effect in women with PPH (The WOMAN trial) is underway. TXA might also affect coagulation factors and platelets.  Objectives. To examine the effect of TXA on thrombin generation, platelet function, fibrinogen, D-dimer and coagulation factors in women with PPH.  Methods. We will conduct a sub-study within the WOMAN trial...
December 15, 2016: Wellcome Open Res
https://www.readbyqxmd.com/read/28088607/transcatheter-aortic-valve-implantation-leads-to-a-restoration-of-von-willebrand-factor-vwf-abnormalities-in-patients-with-severe-aortic-stenosis-incidence-and-relevance-of-clinical-and-subclinical-vwf-dysfunction-in-patients-undergoing-transfemoral-tavi
#5
Alexander Sedaghat, Hannah Kulka, Jan-Malte Sinning, Nora Falkenberg, Julia Driesen, Barbara Preisler, Christoph Hammerstingl, Georg Nickenig, Bernd Pötzsch, Johannes Oldenburg, Hans-Jörg Hertfelder, Nikos Werner
BACKGROUND: In this study, we sought to analyze the incidence and relevance of von Willebrand factor (VWF) abnormalities in patients undergoing transcatheter aortic valve implantation (TAVI), especially on perioperative bleeding. Furthermore, we hypothesized that, similar to aortic valve surgery, TAVI results in a restoration of VWF abnormalities. METHODS AND RESULTS: We performed a prospective analysis of periinterventional VWF parameters in 74 patients (80±7years, female in 37...
January 7, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28087247/activity-of-von-willebrand-factor-and-levels-of-vwf-cleaving-protease-adamts13-in-preterm-and-full-term-neonates
#6
T Strauss, N Elisha, B Ravid, N Rosenberg, A Lubetsky, S Levy-Mendelovich, I Morag, U Nowak Göttl, G Kenet
Von Willebrand Factor (VWF) has a central role in primary hemostasis. Its biological activity is related to the size of VWF multimers, spontaneously binding to platelets and inducing circulating microthrombi formation. This process is down-regulated by the VWF cleaving protease ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin motif). To date, information regarding the levels of ADAMTS13 in neonates and preterm infants is scarce. Our aim was to study ADAMTS13, VWF antigen (Ag) and Ristocetin cofactor (RiCof) activity in neonates and evaluate potential correlations with perinatal complications...
December 29, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28076816/mutation-g1629e-increases-von-willebrand-factor-cleavage-via-a-cooperative-destabilization-mechanism
#7
Camilo Aponte-Santamaría, Svenja Lippok, Judith J Mittag, Tobias Obser, Reinhard Schneppenheim, Carsten Baldauf, Frauke Gräter, Ulrich Budde, Joachim O Rädler
The large multimeric glycoprotein von Willebrand Factor (VWF) plays a pivotal adhesive role during primary hemostasis. VWF is cleaved by the protease ADAMTS13 as a down-regulatory mechanism to prevent excessive VWF-mediated platelet aggregation. For each VWF monomer, the ADAMTS13 cleavage site is located deeply buried inside the VWF A2 domain. External forces in vivo or denaturants in vitro trigger the unfolding of this domain, thereby leaving the cleavage site solvent-exposed and ready for cleavage. Mutations in the VWF A2 domain, facilitating the cleavage process, cause a distinct form of von Willebrand disease (VWD), VWD type 2A...
January 10, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28071872/mutant-botrocetin-2-inhibits-von-willebrand-factor-induced-platelet-agglutination
#8
T Matsui, A Hori, J Hamako, F Matsushita, Y Ozeki, Y Sakurai, M Hayakawa, M Matsumoto, Y Fujimura
BACKGROUND: Botrocetin-2 (Bot2) is a botrocetin-like protein composed of α and β subunits that have been cloned from the snake Bothrops jararaca. Bot2 binds specifically to von Willebrand factor (VWF), and the complex induces glycoprotein (GP) Ib-dependent platelet agglutination. OBJECTIVES: We exploited Bot2's VWF-binding capacity to try and create a mutant Bot2 that binds to VWF but inhibits platelet agglutination. METHODS AND RESULTS: Several point mutations were introduced into Bot2 cDNA, and the recombinant protein (rBot2) was purified on an anti-botrocetin column...
January 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28067100/effects-of-a-single-bout-of-strenuous-exercise-on-platelet-activation-in-female-apoe-ldlr-mice
#9
K Przyborowski, H Kassassir, M Wojewoda, K Kmiecik, B Sitek, K Siewiera, A Zakrzewska, A M Rudolf, R Kostogrys, C Watala, J A Zoladz, S Chlopicki
Strenuous physical exercise leads to platelet activation that is normally counterbalanced by the production of endothelium-derived anti-platelet mediators, including prostacyclin (PGI2) and nitric oxide (NO). However, in the case of endothelial dysfunction, e.g. in atherosclerosis, there exists an increased risk for intravascular thrombosis during exercise that might be due to an impairment in endothelial anti-platelet mechanisms. In the present work, we evaluated platelet activation at rest and following a single bout of strenuous treadmill exercise in female ApoE/LDLR(-)(/-) mice with early (3-month-old) and advanced (7-month-old) atherosclerosis compared to female age-matched WT mice...
January 9, 2017: Platelets
https://www.readbyqxmd.com/read/28065360/intracavernous-injection-of-human-umbilical-cord-blood-mononuclear-cells-improves-erectile-dysfunction-in-streptozotocin-induced-diabetic-rats
#10
Tugba Cengiz, Ecem Kaya, Didem Yilmaz Oral, Isil Ozakca, Nur Bayatli, Arzu Zeynep Karabay, Tugba Altun Ensari, Tuna Karahan, Enis Yilmaz, Serap Gur
INTRODUCTION: Erectile dysfunction (ED) worsens in men with diabetes. Human umbilical cord blood (HUCB), because of its widespread availability and low immunogenicity, is a valuable source for stem cell-based therapies. AIM: To determine the effect of intracavernous injection of HUCB mononuclear cells (MNCs) on ED in rats with diabetes induced by streptozotocin. METHODS: Thirty adult male Sprague-Dawley rats were equally divided into three groups: (i) control, (ii) diabetes induced by streptozotocin (35 mg/kg intravenously for 8 weeks), and (iii) diabetic rats treated with MNCs (1 × 10(6) cells by intracavernosal injection)...
January 2017: Journal of Sexual Medicine
https://www.readbyqxmd.com/read/28064200/rare-variants-in-gp1bb-are-responsible-for-autosomal-dominant-macrothrombocytopenia
#11
Suthesh Sivapalaratnam, Sarah K Westbury, Jonathan C Stephens, Daniel Greene, Kate Downes, Anne M Kelly, Claire Lentaigne, William J Astle, Eric G Huizinga, Paquita Nurden, Sofia Papadia, Kathelijne Peerlinck, Christopher J Penkett, David J Perry, Catherine Roughley, Ilenia Simeoni, Kathleen Stirrups, Daniel P Hart, R Campbell Tait, Andrew D Mumford, Nihr BioResource, Michael A Laffan, Kathleen Freson, Willem H Ouwehand, Shinji Kunishima, Ernest Turro
The von Willebrand receptor complex, which is composed of the glycoproteins Ibα and Ibβ, V and IX, plays an essential role in the earliest steps in hemostasis. Over the last four decades, it has become apparent that loss of function of any one of three of the genes encoding these glycoproteins namely, GP1BA, GP1BB and GP9, leads to autosomal recessive macrothrombocytopenia complicated by bleeding. A small number of variants in GP1BA have been reported to cause a milder and dominant form of macrothrombocytopenia but only two tentative reports exists of such a variant in GP1BB By analyzing data from a collection of over 1,000 genome-sequenced patients with a rare bleeding and/or platelet disorder, we have identified a significant association between rare monoallelic variants in GP1BB and macrothrombocytopenia...
November 14, 2016: Blood
https://www.readbyqxmd.com/read/28062498/novel-thrombotic-function-of-a-human-snp-in-stxbp5-revealed-by-crispr-cas9-gene-editing-in-mice
#12
Qiuyu Martin Zhu, Kyung Ae Ko, Sara Ture, Michael A Mastrangelo, Ming-Huei Chen, Andrew D Johnson, Christopher J O'Donnell, Craig N Morrell, Joseph M Miano, Charles J Lowenstein
OBJECTIVE: To identify and characterize the effect of a SNP (single-nucleotide polymorphism) in the STXBP5 locus that is associated with altered thrombosis in humans. GWASs (genome-wide association studies) have identified numerous SNPs associated with human thrombotic phenotypes, but determining the functional significance of an individual candidate SNP can be challenging, particularly when in vivo modeling is required. Recent GWAS led to the discovery of STXBP5 as a regulator of platelet secretion in humans...
December 29, 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28060126/pediatric-acquired-von-willebrand-syndrome-in-cardiopulmonary-disorders-do-laboratory-abnormalities-predict-bleeding-risk
#13
Saman K Hashmi, Mireya P Velasquez, Donald L Yee, Shiu-Ki Hui, Donald Mahoney, Lakshmi V Srivaths
There are conflicting reports on whether or not laboratory abnormalities in pediatric acquired von Willebrand syndrome (AVWS) predict bleeding manifestations in patients with cardiopulmonary disorders (CPD). We retrospectively reviewed charts of patients with AVWS and CPD (n=16) seen at Texas Children's Hospital from 2003 to 2012. The most common CPD were valve stenoses, ventricular septal defects, and pulmonary hypertension. All patients had loss of high molecular weight multimers. Fifteen (94%) patients presented with bleeding symptoms, with menorrhagia and epistaxis being the most common...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28060120/type-2b-von-willebrand-disease-an-unusual-cause-of-severe-neonatal-thrombocytopenia
#14
Seth J Rotz, Joseph S Palumbo, Russell E Ware
An infant with presumed maternal immune thrombocytopenic purpura had persistent thrombocytopenia with platelet clumping. The patient had no significant bleeding symptoms in the first year of life and von Willebrand antigen and ristocetin cofactor activity were normal. Absent high molecular weight multimers ultimately led to a genetically proven diagnosis of type 2B von Willebrand disease (3964G>A VWF exon 28), highlighting the challenges of establishing this diagnosis in infants.
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28059438/optimized-method-for-isolating-highly-purified-and-functional-porcine-aortic-endothelial-and-smooth-muscle-cells
#15
Farideh Beigi, Mitalben Patel, Marco A Morales-Garza, Caitlin Winebrenner, Andrea S Gobin, Eric Chau, Luiz C Sampaio, Doris A Taylor
BACKGROUND: Numerous protocols exist for isolating aortic endothelial and smooth muscle cells from small animals. However, establishing a protocol for isolating pure cell populations from large animal vessels that are more elastic has been challenging. We developed a simple sequential enzymatic approach to isolate highly purified populations of porcine aortic endothelial and smooth muscle cells. METHODS: The lumen of a porcine aorta was filled with 25 U/mL dispase solution and incubated at 37°C to dissociate the endothelial cells...
January 6, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28057741/factor-viii-v-c-domain-swaps-reveal-discrete-c-domain-roles-in-factor-viii-function-and-intracellular-trafficking
#16
Eduard H T M Ebberink, Eveline A M Bouwens, Esther Bloem, Mariëtte Boon-Spijker, Maartje Van den Biggelaar, Jan Voorberg, Alexander B Meijer, Koen Mertens
Factor VIII C-domains are believed to contain specific function in co-factor activity and in interaction with von Willebrand factor. We have previously shown that factor VIII is co-targeted with von Willebrand factor to the Weibel-Palade bodies in blood outgrowth endothelial cells, even when factor VIII carries mutations in the light chain that are associated with defective von Willebrand factor binding. In this study we furthermore addressed the contribution of individual factor VIII C-domains in intracellular targeting, von Willebrand factor binding and co-factor activity by factor VIII/V C-domain swapping...
January 5, 2017: Haematologica
https://www.readbyqxmd.com/read/28055145/conformational-quiescence-of-adamts13-prevents-proteolytic-promiscuity-comment
#17
W J Cao, X L Zheng
Von Willebrand factor (VWF) is the only substrate identified for ADAMTS13 to date [1]. How ADAMTS13 achieves such remarkable substrate specificity remains a mystery. Previous studies demonstrated that carboxyl-terminal domains including 2-8(th) thrombospondin type 1 repeat (TSP1) and CUB domains are dispensable for cleavage of VWF substrate under various conditions [2, 3]. However, these carboxyl-terminal domains were later found to exhibit a disulfide bond reducing activity [4] and to interact with globular VWF via its D4 domain [5]...
January 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28043678/associations-between-blood-coagulation-markers-nt-probnp-and-risk-of-incident-heart-failure-in-older-men-the-british-regional-heart-study
#18
S Goya Wannamethee, Peter H Whincup, Olia Papacosta, Lucy Lennon, Gordon D Lowe
AIMS: Chronic heart failure (HF) is associated with activation of blood coagulation but there is a lack of prospective studies on the association between coagulation markers and incident HF in general populations. We have examined the association between the coagulation markers fibrinogen, von Willebrand Factor (VWF), Factors VII, VIII and IX, D-dimer, activated protein C (APC) and activated partial thromboplastin time (aPPT) with NT-proBNP and incident HF. METHODS AND RESULTS: Prospective study of 3366 men aged 60-79years with no prevalent HF, myocardial infarction or venous thrombosis and who were not on warfarin, followed up for a mean period of 13years, in whom there were 203 incident HF cases...
December 20, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28035064/functional-assessment-of-von-willebrand-factor-expression-by-cancer-cells-of-non-endothelial-origin
#19
Anahita Mojiri, Konstantin Stoletov, Maria Areli Lorenzana Carrillo, Lian Willetts, Saket Jain, Roseline Godbout, Paul Jurasz, Consolato M Sergi, David D Eisenstat, John D Lewis, Nadia Jahroudi
Von Willebrand factor (VWF) is a highly adhesive procoagulant molecule that mediates platelet adhesion to endothelial and subendothelial surfaces. Normally it is expressed exclusively in endothelial cells (ECs) and megakaryocytes. However, a few studies have reported VWF detection in cancer cells of non-endothelial origin, including osteosarcoma. A role for VWF in cancer metastasis has long been postulated but evidence supporting both pro- and anti-metastatic roles for VWF has been presented. We hypothesized that the role of VWF in cancer metastasis is influenced by its cellular origin and that cancer cell acquisition of VWF expression may contribute to enhanced metastatic potential...
December 27, 2016: Oncotarget
https://www.readbyqxmd.com/read/28029003/effect-of-vitamin-d-status-on-von-willebrand-factor-and-adamts13-in-diabetic-patients-on-chronic-hemodialysis
#20
Keren Cohen-Hagai, Gloria Rashid, Yael Einbinder, Meital Ohana, Sydney Benchetrit, Tali Zitman-Gal
Von Willebrand factor (vWF) is a glycoprotein with a crucial role in the formation of platelet thrombi, and ADAMTS13 is the main enzyme responsible for vWF cleavage. Both are important in the relationship between diabetic nephropathy, hypercoagulability, and cardiovascular disease. This study evaluated a potential relationship between vitamin D (vitD) levels, vWF, ADAMTS13 activity, and inflammation in diabetic patients on chronic hemodialysis (HD). Blood samples from 52 diabetic patients on chronic HD were obtained to determine vitD levels, vWF, and ADAMTS13 activity, and inflammatory markers...
March 2017: Annals of Laboratory Medicine
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