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https://www.readbyqxmd.com/read/29453814/preclinical-evaluation-of-a-semi-automated-and-rapid-commercial-electrophoresis-assay-for-von-willebrand-factor-multimers
#1
Marika Pikta, Galina Zemtsovskaja, Hector Bautista, Georges Nouadje, Timea Szanto, Margus Viigimaa, Valdas Banys
BACKGROUND: The von Willebrand factor (VWF) multimer test is required to correctly subtype qualitative type 2 von Willebrand disease (VWD). The current VWF multimer assays are difficult, nonstandardized, and time-consuming. The purpose of this study was to evaluate the clinical utility of the commercial VWF multimer kit by Sebia (Lisses, France), an electrophoresis technique yielding same-day results. METHODS: Ten healthy volunteer plasma samples, in-house reference plasma (IRP) and commercial normal plasma (CNP) samples, 10 plasma samples from patients with a known VWD type, 1 hemophilia A plasma sample, and 7 external quality assurance (EQA) samples were analyzed using the commercial VWF multimer kit...
February 17, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29447730/no-163-gynaecological-and-obstetric-management-of-women-with-inherited-bleeding-disorders
#2
Christine Demers, Christine Derzko, Michele David, Joanne Douglas
OBJECTIVE: The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in adolescents presenting with menorrhagia. OPTIONS: Diagnostic tools and specific medical and, where appropriate, surgical alternatives to management are reviewed and evidence-based recommendations presented. EVIDENCE: A MEDLINE search of the English literature between January 1975 and November 2003 was performed using the following key words: menorrhagia, uterine bleeding, pregnancy, von Willebrand, congenital bleeding disorder, desmopressin/DDAVP, tranexamic acid, oral contraceptives, medroxyprogesterone, therapy, hysterectomy, anesthesia, epidural, spinal...
February 2018: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/29446520/lenalidomide-as-a-novel-therapy-for-gastrointestinal-angiodysplasia-in-von-willebrand-disease
#3
N V Khatri, B Patel, D R Kohli, S S Solomon, K Bull-Henry, C M Kessler
INTRODUCTION: Lenalidomide is a thalidomide analog with anti-angiogenic properties. Previous case reports suggest its efficacy in preventing gastrointestinal bleeding (GIB) secondary to angiodysplasia (AD) in hereditary haemorrhagic telangiectasia and potentially in reversing AD. We present the first case series to explore lenalidomide as a treatment for AD-related GIB in patients with von Willebrand disease (VWD). METHODS: A retrospective chart review was conducted to include patients with VWD, who were evaluated from 2010 to 2013 and who had received lenalidomide to treat recurrent GIB secondary to AD...
February 15, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29427305/comprehensive-re-evaluation-of-historical-von-willebrand-disease-diagnosis-in-association-with-whole-blood-platelet-aggregation-and-function
#4
V Nummi, R Lassila, L Joutsi-Korhonen, E Armstrong, T Szanto
INTRODUCTION: Diagnosis of von Willebrand disease (VWD) is challenging, particularly for type 1. The current diagnostic guidelines emphasize simultaneous bleeding symptoms and von Willebrand factor (VWF) levels of <30-40 IU/dL. Historical diagnoses require updated evaluation. We assessed the accuracy of past VWD diagnoses in our comprehensive care center with the standardized bleeding score (BS) and central laboratory analysis, focusing on VWF-dependent platelet functions in whole blood...
February 10, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29403324/pharmacokinetics-based-clinical-management-of-acquired-von-willebrand-syndrome-a-case-report
#5
Candice M Baldeo, Candido E Rivera, Han W Tun, Prakash Vishnu
von Willebrand disease (VWD) is a common bleeding disorder caused by defective or low levels of von Willebrand factor (VWF). Although most cases of VWD are caused by genetic mutations, some are acquired due to various disease states. In managing VWD, the aim is to normalize plasma levels of both VWF and factor VIII (FVIII), as this aids in hemostasis. Desmopressin usually corrects VWF level in type 1 VWD by inducing the release of endogenous VWF. In cases where desmopressin is ineffective or cannot be used, transfusion of virally inactivated, plasma-derived VWF/FVIII concentrate or infusion of recombinant VWF (Vonvendi) is indicated...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29389944/genetic-variation-in-the-c-type-lectin-receptor-clec4m-in-type-1-von-willebrand-disease-patients
#6
Eric Manderstedt, Christina Lind-Halldén, Stefan Lethagen, Christer Halldén
von Willebrand factor (VWF) levels in healthy individuals and in patients with type 1 von Willebrand disease (VWD) are influenced by genetic variation in several genes, e.g. VWF, ABO, STXBP5 and CLEC4M. This study aims to screen comprehensively for CLEC4M variants and investigate their association with type 1 VWD in the Swedish population. In order to screen for CLEC4M variants, the CLEC4M gene region was re-sequenced and the polymorphic neck region was genotyped in 106 type 1 VWD patients from unrelated type 1 VWD families...
2018: PloS One
https://www.readbyqxmd.com/read/29388750/high-proportion-of-patients-with-bleeding-of-unknown-cause-in-persons-with-a-mild-to-moderate-bleeding-tendency-results-from-the-vienna-bleeding-biobank-vibb
#7
J Gebhart, S Hofer, S Panzer, P Quehenberger, R Sunder-Plassmann, G Hoermann, E Eigenbauer, H Haslacher, S Kepa, P A Kyrle, S Eichinger, P Knöbl, L Eischer, C Mannhalter, C Ay, I Pabinger
INTRODUCTION: Data on clinical characteristics and the prevalence of underlying coagulopathies in patients with mild-to-moderate bleeding disorders (MBDs) are scarce. AIM: We established the Vienna Bleeding Biobank (VIBB) to characterize and thoroughly investigate Austrian patients with MBDs. RESULTS: Four hundred eighteen patients (female = 345, 82.5%) were included. A platelet function defect (PFD) was diagnosed in 26 (6.2%) and a possible PFD in 30 (7...
February 1, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29378695/how-i-treat-type-2b-von-willebrand-disease
#8
Rebecca Kruse-Jarres, Jill M Johnsen
Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder caused by changes in von Willebrand factor (VWF) that enhance binding of VWF to GPIb on platelets. Although this disorder is seemingly well defined due to this single molecular defect, in reality type 2B VWD is a clinically heterogeneous disorder that can be difficult to identify and manage. Diagnostic criteria include a history of mucocutaneous bleeding, laboratory studies showing enhanced VWF binding of platelets and/or a 2B VWD genetic variant, and a family history consistent with autosomal dominant inheritance...
January 29, 2018: Blood
https://www.readbyqxmd.com/read/29378356/a-mechanistic-model-to-quantify-von-willebrand-factor-release-survival-and-proteolysis-in-patients-with-von-willebrand-disease
#9
Myriam Ferrari, Federico Galvanin, Massimiliano Barolo, Viviana Daidone, Roberto Padrini, Fabrizio Bezzo, Alessandra Casonato
A reduced von Willebrand factor (VWF) synthesis or survival, or its increased proteolysis, alone or in combination, contributes to the development of von Willebrand disease (VWD).We describe a new, simple mechanistic model for exploring how VWF behaves in well-defined forms of VWD after its 1-desamino-8-D-arginine vasopressin (DDAVP)-induced release from endothelial cells. We aimed to ascertain whether the model can consistently predict VWF kinetic changes. The study involved 9 patients with VWD types Vicenza (a paradigmatic form with a reduced VWF survival), 8 type 2B, 2 type 2A-I, 1 type 2A-II (associated with an increased VWF proteolysis), and 42 normal controls, whose VWF levels were measured after a 24-hour-long DDAVP test...
February 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29370241/is-gingival-bleeding-a-symptom-of-type-2-and-3-von-willebrand-disease
#10
Lisa Epping, Wolfgang Miesbach, Katrin Nickles, Peter Eickholz
BACKGROUND: Von Willebrand disease (VWD) is the most common inherent bleeding disorder. Gingival bleeding is a frequently reported symptom of VWD. However, gingival bleeding is also a leading symptom of plaque-induced gingivitis and untreated periodontal disease. In type 1 VWD gingival bleeding was not increased compared to controls. Thus, this study evaluated whether type 2 and 3 VWD determines an increased susceptibility to gingival bleeding in response to the oral biofilm. METHODS: Twenty-four cases and 24 controls matched for age, sex, periodontal diagnosis, number of teeth and smoking were examined hematologically (VWF antigen, VWF activity, factor VIII activity) and periodontally (Gingival Bleeding Index [GBI]), bleeding on probing [BOP], Plaque Control Record [PCR], periodontal inflamed surface area [PISA], vertical probing attachment level)...
2018: PloS One
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/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
#12
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/29304535/circulating-angiogenic-mediators-in-patients-with-moderate-and-severe-von-willebrand-disease-a-multicentre-cross-sectional-study
#13
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/29296853/role-of-calcium-in-regulating-the-intra-and-extracellular-cleavage-of-von-willebrand-factor-by-the-protease-adamts13
#14
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/29296712/antifibrinolytic-therapy-for-preventing-vwd-related-postpartum-hemorrhage-indications-and-limitations
#15
Peter A Kouides
Publisher's Note: This article has a companion Counterpoint by Ragni. 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
#16
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/29285851/von-willebrand-factor-propeptide-to-antigen-ratio-identifies-platelet-activation-and-reduced-vwf-survival-phenotype-in-mice
#17
Paula M Jacobi, Sachiko Kanaji, David Jakab, Ashley L Gehrand, Jill M Johnsen, Sandra L Haberichter
BACKGROUND: Reduced plasma survival of VWF is characteristic of patients with type 1C VWD. These subjects can be identified by an increased steady-state ratio of plasma VWFpp to VWF:Ag. A similar phenotype occurs in mice with the Mvwf1 allele. OBJECTIVES: To 1) determine if VWFpp/VWF:Ag ratio can be used to identify a "type 1C" phenotype in mice, 2) determine the most reliable method for murine blood sampling, and 3) identify the source of VWF released during problematic blood collection...
December 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29251812/the-physical-spacing-between-the-von-willebrand-factor-d-d3-and-a1-domains-regulates-platelet-adhesion-in-vitro-and-in-vivo
#18
Changjie Zhang, Anju Kelkar, Mehrab Nasirikenari, Joseph T Y Lau, Michele Sveinsson, Umesh C Sharma, Saraswati Pokharel, Sriram Neelamegham
BACKGROUND: Previous ex vivo studies using truncated VWF (Von Willebrand Factor) suggest that domain-level molecular architecture may control platelet-GpIbα binding function. OBJECTIVE: We determined if this is the case with multimeric VWF in vivo. METHODS: Full-length human VWF ('hV') was modified with a 22-amino acid mucinous stretch at either the N-terminus of VWF-A1 to create 'hNV' or C-terminus to yield 'hCV'. This extends the physical distance between VWF-A1 and the adjacent domains by ~6nm...
December 18, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29222282/advances-in-the-diagnosis-and-treatment-of-von-willebrand-disease
#19
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
#20
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
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