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Von willebrands disease

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https://www.readbyqxmd.com/read/29322496/wt1-peptide-based-immunotherapy-for-advanced-thymic-epithelial-malignancies
#1
Yusuke Oji, Masayoshi Inoue, Yoshito Takeda, Naoki Hosen, Yasushi Shintani, Manabu Kawakami, Takuya Harada, Yui Murakami, Miki Iwai, Mari Fukuda, Sumiyuki Nishida, Jun Nakata, Yoshiki Nakae, Satoshi Takashima, Toshiaki Shirakata, Hiroko Nakajima, Kana Hasegawa, Hiroshi Kida, Takashi Kijima, Soyoko Morimoto, Fumihiro Fujiki, Akihiro Tsuboi, Eiichi Morii, Satoshi Morita, Junichi Sakamoto, Atsushi Kumanogoh, Yoshihiro Oka, Meinoshin Okumura, Haruo Sugiyama
Thymic epithelial tumors are rare malignancies, and no optimal therapeutic regimen has been defined for patients with advanced disease. Patients with advanced thymic epithelial tumors, which were resistant or intolerable to prior therapies, were eligible for this study. Patients received 9 mer-WT1-derived peptide emulsified with Montanide ISA51 adjuvant via intradermal administration once a week as a monotherapy. After the 3 month-protocol treatment, the treatment was continued mostly at intervals of 2 to 4 weeks until disease progression or intolerable adverse events occurred...
January 11, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29319374/sensitive-and-specific-assessment-of-recombinant-von-willebrand-factor-in-platelet-function-analyzer
#2
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/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
#3
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/29306249/biomarker-and-shear-stress-in-secondary-pediatric-pulmonary-hypertension
#4
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
https://www.readbyqxmd.com/read/29304535/circulating-angiogenic-mediators-in-patients-with-moderate-and-severe-von-willebrand-disease-a-multicentre-cross-sectional-study
#5
Dafna J Groeneveld, Yvonne V Sanders, Jelle Adelmeijer, Evelien P Mauser-Bunschoten, Johanna G van der Bom, Marjon H Cnossen, Karin Fijnvandraat, Britta A P Laros-van Gorkom, Karina Meijer, Ton Lisman, Jeroen Eikenboom, Frank W G Leebeek
Inhibition of von Willebrand factor (VWF) expression in endothelial cells results in enhanced, possible dysfunctional angiogenesis, consistent with observations of severe gastrointestinal bleedings caused by vascular malformations in patients with von Willebrand disease (VWD). VWF is stored in endothelial Weibel-Palade bodies (WPB) with several other mediators of angiogenesis, like angiopoietin-2, osteoprotegerin and galectin-3. Increased release of angiopoietin-2 has been observed in medium of endothelial cells lacking VWF, but data on circulating levels of angiogenic factors in patients with VWD are lacking...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29304532/platelet-count-adamts13-activity-von-willebrand-factor-level-and-survival-in-patients-with-colorectal-cancer-5-year-follow-up-study
#6
Nóra Garam, Éva Maláti, György Sinkovits, Tímea Gombos, Attila Szederjesi, Loránd Barabás, László Gráf, Judit Kocsis, Zoltán Prohászka
Distant metastasis is a major cause of colorectal cancer-related death, but the mechanism of tumour progression is not fully understood. There is growing evidence of an interaction between tumour cells and platelets which may influence tumour progression and metastasis formation. Quality and quantity of von Willebrand factor may regulate the interaction between tumour cells and platelets. Our aim was to measure the platelet count, von Willebrand factor antigen (VWF:Ag) levels and ADAMTS13 activity in a large (n = 232) cohort of colorectal cancer patients and to examine their relationships with the stage of the disease and 5-year survival without thrombotic complications using multivariable models...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29304525/low-self-rated-health-is-related-to-blood-hypercoagulability-in-patients-admitted-with-acute-myocardial-infarction
#7
Roland von Känel, Rebecca E Meister-Langraf, Jean-Paul Schmid, Jürgen Barth, Hansjörg Znoj, Ulrich Schnyder, Mary Princip
Self-rated health (SRH) is independently associated with all-cause mortality and adverse cardiovascular outcomes in individuals with and without cardiovascular disease. We examined whether SRH relates to haemostatic factors of a hypercoagulable state with prognostic impact in patients admitted with acute myocardial infarction (MI). We assessed 190 patients (median age: 59 years; all Caucasian; 83% men) within 48 hours of an acute coronary intervention in terms of demographic factors, medical and psychiatric comorbidity, health behaviours, cardiac-related variables and psychosocial characteristics...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29297854/flow-induced-adhesion-of-shear-activated-polymers-to-a-substrate
#8
Masoud Hoore, Kathrin Rack, Dmitry Fedosov, Gerhard Gompper
Adhesion of polymers and proteins to substrates plays a crucial role in many technological applications and biological processes. A prominent example is the von Willebrand factor (VWF) protein, which is essential in blood clotting as it mediates adhesion of blood platelets to the site of injury at high shear rates. VWF is activated by flow and is able to bind efficiently to damaged vessel walls even under extreme flow-stress conditions; however, its adhesion is reversible when the flow strength is significantly reduced or the flow is ceased...
January 3, 2018: Journal of Physics. Condensed Matter: An Institute of Physics Journal
https://www.readbyqxmd.com/read/29296853/role-of-calcium-in-regulating-the-intra-and-extracellular-cleavage-of-von-willebrand-factor-by-the-protease-adamts13
#9
Shobhit Gogia, Anju Kelkar, Changjie Zhang, Kannayakanahalli M Dayananda, Sriram Neelamegham
von Willebrand factor (VWF) and the metalloprotease a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) are present both within endothelial cells (ECs) and in peripheral blood. Calcium concentrations are lower in intracellular compartments (80-400 μM) compared with the extracellular milieu (∼1.25 mM). Because low calcium favors VWF A2-domain proteolysis by ADAMTS13, the dependence of proteolysis rates on calcium was assayed both within ECs and in blood. Confocal microscopy studies demonstrate partial perinuclear colocalization of VWF with ADAMTS13 in human umbilical vein ECs (HUVECs)...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296713/blood-volume-based-von-willebrand-factor-to-prevent-postpartum-hemorrhage-in-von-willebrand-disease
#10
Margaret V Ragni
Publisher's Note: This article has a companion Point by Kouides. Publisher's Note: Join in the discussion of these articles at Blood Advances Community Conversations.
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29288565/high-and-long-term-von-willebrand-factor-expression-after-sleeping-beauty-transposon-mediated-gene-therapy-in-a-mouse-model-of-severe-von-willebrand-disease
#11
Irina Portier, Karen Vanhoorelbeke, Sebastien Verhenne, Inge Pareyn, Nele Vandeputte, Hans Deckmyn, Daniel S Goldenberg, Himanshu B Samal, Manvendra Singh, Zoltán Ivics, Zsuzsanna Izsvák, Simon F De Meyer
BACKGROUND: Type 3 von Willebrand disease (VWD) is characterized by complete absence of von Willebrand factor (VWF). Current therapy is limited to treatment with exogenous VWF/FVIII products, which only provide a short-term solution. Gene therapy offers the potential for a long-term treatment for VWD. OBJECTIVES: To develop an integrative Sleeping Beauty (SB) transposon-mediated VWF gene transfer approach in a preclinical mouse model of severe VWD. METHODS: We established a robust platform for sustained transgene murine (m)VWF expression in the liver of Vwf-/- mice by combining a liver-specific promoter with a sandwich transposon design and the SB100X transposase via hydrodynamic gene delivery...
December 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29285810/von-willebrand-factor-revisited
#12
Senan J Yasar, Obai Abdullah, William Fay, Sudarshan Balla
Degenerative aortic valve stenosis is the most common valvular heart disease seen in developed countries. Since the implementation of transcatheter aortic valve replacement, there has been a surge of efforts to improve procedural technique as well as reduce mortality. Until recently, the mainstay method of assessment of valve implantation as well as paravalvular regurgitation has relied heavily on imaging techniques including transesophageal echocardiography and aortic root angiography. There has been a lot of scientific inquiry to identify biomarkers in the management of patients with aortic stenosis...
December 28, 2017: Journal of Interventional Cardiology
https://www.readbyqxmd.com/read/29258201/impact-of-unsaturated-fatty-acids-on-cytokine-driven-endothelial-cell-dysfunction
#13
Simon Trommer, Anja Leimert, Michael Bucher, Julia Schumann
Polyunsaturated fatty acids (PUFA) are reported to exert prophylactic and acute therapeutic effects in diseases linked to endothelial dysfunction. In the present study, the consequences of a PUFA enrichment of endothelial cells (cell line TIME) on cell viability, expression of the cytokines interleukin-6 (IL-6), interleukin-8 (IL-8), granulocyte-macrophage colony-stimulating factor (GM-CSF), and monocyte chemoattractant protein 1 (MCP-1), synthesis of the adhesion molecules intercellular adhesion molecule 1 (ICAM-1) and vascular adhesion molecule 1 (VCAM-1), and production of the coagulation factors plasminogen activator inhibitor-1 (PAI-1), von Willebrand factor (vWF), and tissue factor (TF) was analyzed in parallel...
December 16, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29230812/the-gut-microbiota-an-emerging-risk-factor-for-cardiovascular-and-cerebrovascular-disease
#14
REVIEW
Stefanie Ascher, Christoph Reinhardt
Commensal gut microbiota have recently been implicated in cardiovascular disease (CVD) and cerebrovascular disease. Atherosclerotic plaque formation depends on the colonization status of the host. In addition to host nutrition and the related microbiota-dependent metabolic changes, activation of innate immune pathways triggers the development of atherosclerosis and supports arterial thrombosis. Gnotobiotic mouse models have uncovered that activation of Toll-like receptor-2 by gut microbial ligands supports von Willebrand factor-integrin mediated platelet deposition to the site of vascular injury...
December 12, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#15
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
https://www.readbyqxmd.com/read/29226875/vegfr1-and-vegfr2-in-alzheimer-s-disease
#16
Rachel Harris, James Scott Miners, Shelley Allen, Seth Love
Vascular endothelial growth factor (VEGF) is a potent angiogenic factor. Despite upregulation of VEGF in the brain in Alzheimer's disease (AD), probably in response to amyloid-β, vasoconstriction, and tissue hypoxia, there is no consequent increase in microvessel density. VEGF binds to and activates VEGF receptor 2 (VEGFR2), but also binds to VEGF receptor 1 (VEGFR1), which exists in less-active membrane-bound and inactive soluble (sVEGFR1) forms and inhibits pro-angiogenic signaling. We have investigated whether altered expression of VEGF receptors might account for the lack of angiogenic response to VEGF in AD...
December 6, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29225984/acquired-von-willebrand-disease-associated-with-monoclonal-gammopathy-of-unknown-significance
#17
Sijan Basnet, Catherine Lin, Rashmi Dhital, Izza Mir, Elan Mohanty, Biswaraj Tharu, Sushil Ghimire, Dilli Ram Poudel
We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29222282/advances-in-the-diagnosis-and-treatment-of-von-willebrand-disease
#18
REVIEW
Ruchika Sharma, Veronica H Flood
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, yet diagnosis and management remain challenging. Development and use of bleeding assessment tools allows for improved stratification of which patients may require further assessment and which patients are most likely to require treatment of their VWD. New options for laboratory assessment of von Willebrand factor (VWF) activity include a new platelet-binding assay, the VWF:GPIbM, which is subject to less variability than the ristocetin cofactor activity assay, and collagen-binding assays that provide insight into a different function of VWF...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29208651/a-12-3-kb-duplication-within-the-vwf-gene-in-pigs-affected-by-von-willebrand-disease-type-3
#19
Stefanie Lehner, Mahnaz Ekhlasi-Hundrieser, Carsten Detering, Hanna Allerkamp, Christiane Pfarrer, Mario von Depka Prondzinski
Von Willebrand Disease (VWD) type 3 is a serious and sometimes fatal hereditary bleeding disorder. In pigs, the disease has been known for decades and affected animals are used as models for the human disease. Due to the recessive mode of inheritance of VWD type 3, severe bleeding is typically seen in homozygous individuals. We sequenced the complete porcine VWF (Von Willebrand Factor) cDNA and detected a tandem duplication of exons 17 and 18, causing a frameshift and a premature termination codon (p.Val814LeufsTer3) in the affected pig...
December 5, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/29208350/efficient-differentiation-of-bone-marrow-mesenchymal-stem-cells-into-endothelial-cells-in%C3%A2-vitro
#20
Chengen Wang, Yuan Li, Min Yang, Yinghua Zou, Huihui Liu, Zeyin Liang, Yue Yin, Guochen Niu, Ziguang Yan, Bihui Zhang
OBJECTIVE: Endothelial cells (ECs) play an important role in neovascularisation, but are too limited in number for adequate therapeutic applications. Mesenchymal stem cells (MSCs) have the potential to differentiate into endothelial lineage cells, which makes them attractive candidates for therapeutic angiogenesis. The aim of this study was to investigate efficient differentiation of MSCs into ECs by inducing medium in vitro. METHODS: MSCs were isolated from bone marrow by density gradient centrifugation...
December 2, 2017: European Journal of Vascular and Endovascular Surgery
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