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https://www.readbyqxmd.com/read/28533135/enhanced-local-disorder-in-a-clinically-elusive-von-willebrand-factor-provokes-high-affinity-platelet-clumping
#1
Alexander Tischer, Venkata R Machha, Juan P Frontroth, Maria A Brehm, Tobias Obser, Reinhard Schneppenheim, Leland Mayne, S Walter Englander, Matthew Auton
Mutation of the cysteines forming the disulfide loop of the platelet GPIbα adhesive A1 domain of von Willebrand factor causes quantitative VWF deficiencies in the blood and von Willebrand disease. We report two cases of transient severe thrombocytopenia induced by DDAVP-treatment. Cys1272Trp and Cys1458Tyr mutations identified by genetic sequencing implicate an abnormal gain-of-function phenotype, evidenced by thrombocytopenia, that quickly relapses back to normal platelet counts and deficient plasma VWF. Using surface plasmon resonance, analytical rheology, and hydrogen-deuterium exchange mass spectrometry (HXMS), we decipher mechanisms of A1-GPIbα mediated platelet adhesion and resolve dynamic secondary structure elements that regulate the binding pathway...
May 19, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/28527913/von-willebrand-factor-deficiency-reduces-liver-fibrosis-in-mice
#2
Nikita Joshi, Anna K Kopec, Jessica L Ray, Holly Cline-Fedewa, Dafna J Groeneveld, Ton Lisman, James P Luyendyk
Liver diseases are associated with complex changes in the hemostatic system and elevated levels of the platelet-adhesive protein Von Willebrand factor (VWF) are reported in patients with acute and chronic liver damage. Although elevated levels of VWF are associated with fibrosis in the general population, the role of VWF in acute and chronic liver injury has not been examined in depth in experimental settings. We tested the hypothesis that VWF deficiency inhibits experimental liver injury and fibrosis. Wild-type (WT) and VWF-deficient mice were challenged with carbon tetrachloride (CCl4) and the impact of VWF deficiency on acute liver injury and chronic liver fibrosis was determined...
May 17, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28526067/the-chimeric-monoclonal-antibody-mhcsz-123-against-human-von-willebrand-factor-a3-domain-inhibits-high-shear-arterial-thrombosis-in-a-rhesus-monkey-model
#3
Shundong Ji, Miao Jiang, Bin Yan, Fei Shen, Yang He, Aini Wan, Lijun Xia, Changgeng Ruan, Yiming Zhao
BACKGROUND: SZ-123, a murine monoclonal antibody that targets the human von Willebrand factor (VWF) A3 domain and blocks the binding of collagen, is a powerful antithrombotic. In a Rhesus monkey model of thrombosis, SZ-123 had no side effects, such as bleeding or thrombocytopenia. METHODS: The mouse/human chimeric version of SZ-123, MHCSZ-123, was developed and maintained inhibitory capacities in vitro and ex vivo after injection into monkeys. CHO-S cells were selected for stable expression of MHCSZ-123...
May 19, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28525200/-von-willebrand-disease-type-3-falsely-diagnosed-as-hemophilia-a-a-case-report
#4
D Benlaldj, M A Moueden, F Seghier
The von Willebrand disease type 3 can be confused with hemophilia A because a very low level of factor VIIIC, we will report the case of patient with von Willebrand disease type 3 treated as moderate hemophilia A.
2017: Revue Médicale de Bruxelles
https://www.readbyqxmd.com/read/28523822/development-of-a-novel-flow-cytometric-immunobead-array-to-quantify-vwf-ag-and-vwf-gpibr-and-its-application-in-acute-myocardial-infarction
#5
Bin Yan, Mengqiao Xu, Yunxiao Zhao, Haijun Guo, Lijun Xia, Changgeng Ruan, Yiming Zhao
OBJECTIVES: Both von Willebrand disease (VWD) and acute myocardial infarction (AMI) involve quantitative and qualitative changes in von Willebrand factor (VWF). Our objective was to develop a rapid and precise flow cytometric immunobead array (FCIA) to quantify VWF antigen (VWF:Ag) and ristocetin-triggered platelet glycoprotein Ib binding (VWF:GPIbR), and apply it in a clinical setting. METHODS: Microbeads, coated with monoclonal antibodies for SZ29 or SZ151 IgG, were incubated with diluted plasma...
May 18, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28521259/von-willebrand-factor-and-its-cleaving-protease-adamts13-balance-in-coronary-artery-vessels-lessons-learned-from-thrombotic-thrombocytopenic-purpura-a-narrative-review
#6
REVIEW
Nuccia Morici, Silvia Cantoni, Francesco Panzeri, Alice Sacco, Chiara Rusconi, Miriam Stucchi, Fabrizio Oliva, Marco Cattaneo
BACKGROUND: Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a microangiopathic syndrome that presents as an acute medical emergency. In this review we will explore the evidence of a two-way relationship between TTP and ACS. Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS. METHODS AND RESULTS: Pubmed, MEDLINE and EMBASE, CINHAL, COCHRANE and Google Scholar databases were searched from inception to January 2017...
May 12, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28520702/vonvendi-recombinant-von-willebrand-factor
#7
(no author information available yet)
No abstract text is available yet for this article.
May 22, 2017: Medical Letter on Drugs and Therapeutics
https://www.readbyqxmd.com/read/28516863/sporadic-creutzfeldt-jakob-disease-in-2-plasma-product-recipients-united-kingdom
#8
Patrick Urwin, Kumar Thanigaikumar, James W Ironside, Anna Molesworth, Richard S Knight, Patricia E Hewitt, Charlotte Llewelyn, Jan Mackenzie, Robert G Will
Sporadic Creutzfeldt-Jakob disease (sCJD) has not been previously reported in patients with clotting disorders treated with fractionated plasma products. We report 2 cases of sCJD identified in the United Kingdom in patients with a history of extended treatment for clotting disorders; 1 patient had hemophilia B and the other von Willebrand disease. Both patients had been informed previously that they were at increased risk for variant CJD because of past treatment with fractionated plasma products sourced in the United Kingdom...
June 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28514969/endothelial-progenitor-cell-number-is-not-decreased-in-34-children-with-juvenile-dermatomyositis-a-pilot-study
#9
Dong Xu, Akadia Kacha-Ochana, Gabrielle A Morgan, Chiang-Ching Huang, Lauren M Pachman
OBJECTIVE: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). METHODS: After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34(+)/VEGFR2(+)/CD45dim(-), and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag)...
May 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28512563/acquired-von-willebrand-syndrome-associated-to-secondary-igm-mgus-emerging-after-autologous-stem-cell-transplantation-for-al-amyloidosis
#10
Hina Qamar, Adrienne Lee, Karen Valentine, Leslie Skeith, Victor H Jimenez-Zepeda
Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#11
Chakri Gavva, Prapti Patel, Yu-Min Shen, Eugene Frenkel, Ravi Sarode
Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency...
April 27, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28511390/expression-of-angiogenic-factors-in-psoriasis-vulgaris
#12
Lakshna Sankar, Dhanalakshmi Arumugam, Sudha Boj, Priyanka Pradeep
INTRODUCTION: Psoriasis is a chronic inflammatory skin disease characterized by epidermal hyperproliferation, abnormal differentiation and inflammatory infiltration in the dermis. The dermal microvascular expansion associated with abnormal orientation and dilatation of capillaries in the biopsies of the psoriatic skin suggest that the disease is dependent on angiogenesis. AIM: To analyze and compare the immunohistochemical expression of angiogenic factors - Vascular Endothelial Growth Factor (VEGF), von Willebrand Factor (vWF) and CD 34 in skin biopsies of psoriasis cases with control skin samples; and to correlate the expression of angiogenic factors with Psoriasis Area and Severity Clinical Index (PASI SCORE)...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28506137/elevation-of-serum-park7-and-il-8-levels-is-associated-with-acute-lung-injury-in-patients-with-severe-sepsis-septic-shock
#13
Xiao-Wei Liu, Tao Ma, Quan Cai, Li Wang, Hong-Wei Song, Zhi Liu
OBJECTIVE: Methods containing only clinical information fail to meet the needs of prediction of acute lung injury (ALI) because of the relatively low positive predictive value. This study aimed to investigate the feasibility of using biomarkers as predictors of ALI in populations with severe sepsis/septic shock and to explore difference among biomarkers after adjustment for potential confounders. METHODS: Serum specimens were collected from patients with severe sepsis/septic shock (n = 172) presented to the emergency department...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28503993/dual-inhibition-of-pi3k-and-mtor-by-vs-5584-suppresses-thrombus-formation
#14
Thomas Später, Isabelle Müller, Hermann Eichler, Michael D Menger, Matthias W Laschke, Emmanuel Ampofo
VS-5584 is a highly selective dual kinase inhibitor which suppresses phosphatidylinositol 3-kinase (PI3K) and mammalian target of rapamycin (mTOR) activity. Because these kinases are crucially involved in primary hemostasis, we herein investigated the effect of this compound on thrombus formation in vitro and in vivo. Pretreatment of washed platelets (WP) or platelet-rich plasma (PRP) with VS-5584 inhibited the agonist-induced activation of surface glycoprotein complex (GP)IIb/IIIa and the upregulation of P-selectin...
May 15, 2017: Platelets
https://www.readbyqxmd.com/read/28503498/evaluation-of-energy-balance-on-human-telomerase-reverse-transcriptase-htert-alternative-splicing-by-semi-quantitative-rt-pcr-in-human-umbilical-vein-endothelial-cells
#15
Mohaddeseh Behjati, Mohammad Hashemi, Mohammad Kazemi, Mansoor Salehi, Shaghayegh Haghjooy Javanmard
BACKGROUND: Decreased high-energy phosphate level is involved in endothelial cell injury and dysfunction. Reduced telomerase activity in endothelial cells in parallel with reduced energy levels might be due to altered direction of alternative splicing machine as a complication of depleted energy during the process of atherosclerosis. MATERIALS AND METHODS: Isolated human umbilical vein endothelial cells (HUVECs) were treated for 24 hours by oligomycine (OM) and 2-deoxy glucose (2-DG)...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28502501/apolipoprotein-b-vs-non-high-density-lipoprotein-cholesterol-association-with-endothelial-hemostatic-markers-and-carotid-intima-media-thickness
#16
David Karasek, Helena Vaverkova, Lubica Cibickova, Jaromira Gajdova, Veronika Kubickova
BACKGROUND: Both apolipoprotein B (apoB) and non-high-density lipoprotein cholesterol (non-HDL-C) are accepted as alternative risk factors or targets for lipid-lowering therapy, which correlate more strongly with cardiovascular events than low-density lipoprotein cholesterol. OBJECTIVE: The aim of this cross-sectional study was to evaluate the differences in plasma levels of plasminogen activator inhibitor-1 (PAI-1) and of von Willebrand factor (vWF) as endothelial hemostatic markers and carotid intima-media thickness (C-IMT) as a morphologic marker for atherosclerotic vascular disease among dyslipidemic individuals with apoB levels higher, estimated or lower based on regression equation of apoB vs non-HDL-C...
March 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28500669/anti-m%C3%A3-llerian-hormone-signalling-is-influenced-by-follistatin-288-but-not-fourteen-other-transforming-growth-factor-beta-superfamily-regulators
#17
Yui Kawagishi, Michael W Pankhurst, Yoshio Nakatani, Ian S McLennan
The hypothesis that, in contrast to other Transforming growth factor-beta (TGFβ) superfamily ligands, the dose-response curve of Anti-Müllerian hormone (AMH) is unmodulated was tested by examining whether known TGFB superfamily modulators affect AMH signalling, using a P19/BRE luciferase reporter assay. AMHC and AMHN,C activated the reporter with an EC50 of approximately 0.5 nM. Follistatins (FS) produced concentration-dependent increases in AMHC - and AMHN,C -inidiated reporter activity, with FS288 being more potent than FS315; however, the maximum bioactivity of AMH was not altered by either follistatin...
May 13, 2017: Molecular Reproduction and Development
https://www.readbyqxmd.com/read/28500622/implementation-of-a-rapid-assay-of-adamts13-activity-was-associated-with-improved-30-day-survival-rate-in-patients-with-acquired-primary-thrombotic-thrombocytopenic-purpura-who-received-platelet-transfusions
#18
Yumi Yoshii, Yoshihiro Fujimura, Charles L Bennett, Ayami Isonishi, Norio Kurumatani, Masanori Matsumoto
BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28499509/ten-year-study-of-postoperative-complications-following-dental-extractions-in-patients-with-inherited-bleeding-disorders
#19
J-T Hsieh, K Klein, M Batstone
Dental extractions challenge the body's haemostatic mechanism. Postoperative bleeding from dental extraction can be prolonged, or even life threatening in patients with inherited bleeding disorders. Pre- and postoperative clotting factor replacements or systemic desmopressin (ddAVP) have been advocated at our institution to prevent bleeding complications in these patients. This study aimed to assess the postoperative bleeding rate in patients with inherited bleeding disorders that underwent dental extractions at our institution between 2003 and 2012...
May 9, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28498508/stroke-in-women-risk-factors-and-clinical-biomarkers
#20
Hamed Mirzaei
Stroke has been emerged as one of major health problems for women worldwide. Increasing of knowledge in this field provided new data in this area which contribute to finding of new risk factors and could improve stroke treatment. A large number studies indicated a variety of risk factors including sex, age, race, smoking, diabetes, and physical inactivity could be involved in stroke in women. The understanding of various aspects involved in women stroke including risk factors, outcomes, stroke recovery, and prevention could provide new therapeutic platforms which could likely lead to better treatment in women stroke...
May 12, 2017: Journal of Cellular Biochemistry
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