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https://www.readbyqxmd.com/read/28696025/a-novel-vwf-variant-associated-with-type-2-von-willebrand-disease-in-german-wirehaired-pointers-and-german-shorthaired-pointers
#1
M Vos-Loohuis, B A van Oost, C Dangel, I Langbein-Detsch, P A Leegwater
Von Willebrand disease (VWD), caused by deficiency of the von Willebrand factor (VWF), is the most common bleeding disorder in humans and dogs. The complete cDNA encoding VWF of a German Wirehaired Pointer with type 2 VWD was sequenced, and we found four variants that alter the amino acid sequence. These variants were: c.1657T>G corresponding to p.Trp553Gly; c.1777G>A (p.Glu593Lys); c.4937A>G (p.Asn1646Ser) and c.5544G>A (p.Met1848Ile). A haplotype of the c.1657G, c.1777A and c.4937G alleles co-segregated with the VWF antigen level in a four-generation pedigree with the disease...
August 2017: Animal Genetics
https://www.readbyqxmd.com/read/28693644/gene-cloning-and-difference-analysis-of-vitellogenin-in-neoseiulus-barkeri-hughes
#2
L Ding, F Chen, R Luo, Q Pan, C Wang, S Yu, L Cong, H Liu, H Li, C Ran
Neoseiulus barkeri (HUGHES) is the natural enemy of spider mites, whiteflies and thrips. Screening for chemically-resistant predatory mites is a practical way to balance the contradiction between the pesticide using and biological control. In this study, the number of eggs laid by fenpropathrin-susceptible and resistant strains of N. barkeri was compared. Additionally, we cloned three N. barkeri vitellogenin (Vg) genes and used quantitative real-time polymerase chain reaction to quantify Vg expression in susceptible and resistant strains...
July 11, 2017: Bulletin of Entomological Research
https://www.readbyqxmd.com/read/28692141/a-discontinuous-autoinhibitory-module-masks-the-a1-domain-of-von-willebrand-factor
#3
Wei Deng, Yingchun Wang, Samuel A Druzak, John F Healey, Anum K Syed, Pete Lollar, Renhao Li
BACKGROUND: How von Willebrand factor (VWF) senses and responds to shear flow remains unclear. In the absence of shear VWF or its fragments can be induced to bind spontaneously to platelet GPIbα. Objectives To elucidate the auto-inhibition mechanism of VWF. METHODS: Hydrogen-deuterium exchange (HDX) of two recombinant VWF fragments expressed from baby hamster kidney cells were measured and compared. RESULTS: The shortA1 protein contains VWF residues 1261-1472 and binds GPIbα with a significantly higher affinity than the longA1 protein that contains VWF residues 1238-1472...
July 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28692112/the-impact-of-bleeding-disorders-on-the-socioeconomic-status-of-adult-patients-results-of-a-comparative-single-centre-cohort-study
#4
Katharina Holstein, Sylvia von Mackensen, Carsten Bokemeyer, Florian Langer
The impact of inherited bleeding disorders on the socioeconomic status (SES) of affected individuals is not clear. The SES of adult patients with congenital bleeding disorders (PWBD) from a centre in Germany (age 42.3 ± 15.0 years) was compared to that of a gender- and age-matched control group of patients with thrombophilia or a thrombotic event (PWT). Patients completed a questionnaire including aspects of SES, impact of the disease on their lives, and health-related quality of life (HRQoL). Forty-five patients were enrolled in each group; 71 % of PBWD had a severe form of the bleeding disorder (FVIII/IX activity < 1 % or VWD type 3), and 60 % of all PWBD were treated on-demand...
July 10, 2017: Hämostaseologie
https://www.readbyqxmd.com/read/28692107/whole-blood-ristocetin-activated-platelet-impedance-aggregometry-multiplate-for-the-rapid-detection-of-von-willebrand-disease
#5
David E Schmidt, Maria Bruzelius, Ammar Majeed, Jacob Odeberg, Margareta Holmström, Anna Ågren
Von Willebrand disease (VWD) is the most common bleeding disorder, but no bedside tests specific for Von Willebrand factor are available. The objective of this study was to evaluate the diagnostic accuracy of whole blood ristocetin-induced platelet aggregometry (WB-RIPA) in VWD. WB-RIPA was performed in VWD patients (n=100) and healthy controls (n=17) using the Multiplate® platelet impedance aggregometry platform. The diagnostic properties of the test were described as sensitivity/specificity, positive and negative predictive value, and ROC area under the curve (AUC)...
July 6, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28691218/safety-of-a-pasteurized-plasma-derived-factor-viii-and-von-willebrand-factor-concentrate-analysis-of-33-years-of-pharmacovigilance-data
#6
Peter Kouides, Kathrin Wawra-Hehenberger, Anna Sajan, Henry Mead, Toby Simon
BACKGROUND: Haemate-P/Humate-P (Humate-P) is a pasteurized human plasma-derived concentrate containing both Factor VIII and von Willebrand factor for treatment of hemophilia A and von Willebrand disease (VWD). STUDY DESIGN AND METHODS: We analyzed the safety of Humate-P based on more than 33 years of postmarketing pharmacovigilance data, representing an estimated exposure of approximately 25,000 patient-years. The analysis comprises reports of potential adverse drug reactions (ADRs) from all sources, reported as part of routine pharmacovigilance at CSL Behring...
July 10, 2017: Transfusion
https://www.readbyqxmd.com/read/28660968/application-of-a-strain-rate-gradient-microfluidic-device-to-von-willebrand-s-disease-screening
#7
Rose J Brazilek, Francisco J Tovar-Lopez, Angus K T Wong, Huyen Tran, Amanda S Davis, James D McFadyen, Zane Kaplan, Sanjeev Chunilal, Shaun P Jackson, Harshal Nandurkar, Arnan Mitchell, Warwick S Nesbitt
Von Willebrand's disease (VWD) is the most common inherited bleeding disorder caused by either quantitative or qualitative defects of von Willebrand factor (VWF). Current tests for VWD require relatively large blood volumes, have low throughput, are time-consuming, and do not incorporate the physiologically relevant effects of haemodynamic forces. We developed a microfluidic device incorporating micro-contractions that harnesses well-defined haemodynamic strain gradients to initiate platelet aggregation in citrated whole blood...
June 29, 2017: Lab on a Chip
https://www.readbyqxmd.com/read/28658773/haemostatic-disorder-in-women-with-unexplained-menorrhagia-a-tertiary-care-centre-experience-from-northern-india
#8
Rashmi Kushwaha, Ashutosh Kumar, Kusum Lata Mishra, Pushp Lata Sankhwar, Renu Singh
INTRODUCTION: Menorrhagia is a common gynaecological problem and its cause remains unexplained in a significant proportion of females. AIM: The present study was done to diagnose a wide range of haemostatic disorders which can give rise to menorrhagia in women of adolescent, postadolescent and perimenopausal age group. MATERIALS AND METHODS: A total of 1100 women presenting to gynaecological emergency with complaints of menorrhagia underwent comprehensive evaluation...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28640903/type-2b-von-willebrand-disease-with-or-without-large-multimers-a-distinction-of-the-two-sides-of-the-disorder-is-long-overdue
#9
Alessandra Casonato, Viviana Daidone, Eva Galletta, Antonella Bertomoro
Most, but not all patients with type 2B von Willebrand disease (VWD)-which features gain-of-function mutations in the A1 domain of von Willebrand factor (VWF)-have no circulating large VWF multimers. Similarities and differences were analysed in 33 type 2B patients, 12 with a normal and 21 with an abnormal multimer pattern, to see whether they should be considered separately. The minimum aggregating dose of ristocetin was similarly reduced in both patient groups, and modulated by their underlying VWF mutations...
2017: PloS One
https://www.readbyqxmd.com/read/28636092/impact-of-diagnosis-of-von-willebrand-disease-on-patient-outcomes-analysis-of-medical-insurance-claims-data
#10
R F Sidonio, K M Haley, D Fallaize
The inherited bleeding disorder von Willebrand disease (VWD) is challenging to diagnose owing to disease heterogeneity, lack of a definitive laboratory test and variations in diagnostic criteria. We evaluated the impact of diagnosis and diagnostic delay on patient outcomes. The PharMetrics Plus Database was interrogated for medical claims for VWD (ICD-9 286.4) and bleeding events between 1 January 2006 and 30 June 2015. Longitudinal analysis was performed of patients newly diagnosed with VWD (≥9 months' continuous enrolment before first VWD claim) through 24 months following diagnosis...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28586149/comparative-analysis-of-von-willebrand-factor-profiles-after-implantation-of-left-ventricular-assist-device-and-total-artificial-heart
#11
H J Reich, J Morgan, F Arabia, L Czer, J Moriguchi, D Ramzy, F Esmailian, L Lam, J Dunhill, O Volod
BACKGROUND: Bleeding remains a challenge during mechanical circulatory support and underlying mechanisms are incompletely understood. Functional von Willebrand Factor (VWF) impairment due to loss of high molecular weight multimers (MWM) produces acquired von Willebrand Disease (VWD) after left ventricular assist device (LVAD). Little is known about VWF multimers with total artificial hearts (TAHs). Here, VWF profiles with LVADs and TAHs are compared using a VWD panel. METHODS: VWD evaluations for patients with LVAD or TAH (2013-14) were retrospectively analyzed and included: VWF activity (Ristocetin Cofactor, VWF:RCo), VWF antigen (VWF:Ag), ratio of VWF:RCo to VWF:Ag, and quantitative VWF multimeric analysis...
June 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28583392/a-novel-molybdenum-disulfide-nanosheet-self-assembled-flower-like-monolithic-sorbent-for-solid-phase-extraction-with-high-efficiency-and-long-service-life
#12
Fanpeng Ran, Hongmei Liu, Xiaoqi Wang, Yong Guo
A novel material consisting of molybdenum disulfide (MoS2) nanosheet that self-assemble into flower-like microspheres which aggregate to form a monolithic matrix with a micro or nano-scaled mesopore structure was successfully synthesized and used as an efficient sorbent for solid-phase extraction (SPE) due to its large specific adsorption area and good stability. The extraction properties of the as-prepared sorbent were evaluated by high-performance liquid chromatography with variable wavelength detection (HPLC-VWD) by analyzing four flavonoids (apigenin, quercetin, luteolin, and kaempferol)...
May 8, 2017: Journal of Chromatography. A
https://www.readbyqxmd.com/read/28581694/abnormal-von-willebrand-factor-secretion-factor-viii-stabilization-and-thrombus-dynamics-in-type-2n-von-willebrand-disease-mice
#13
Laura L Swystun, Ilinca Georgescu, Jeff Mewburn, Meghan Deforest, Kate Nesbitt, Kassandra Hebert, Courtney Dwyer, Christine Brown, Colleen Notley, David Lillicrap
BACKGROUND: von Willebrand factor (VWF) and factor VIII (FVIII) circulate as a non-covalent complex with VWF serving as the carrier for FVIII. VWF indirectly influences secondary hemostasis by stabilizing FVIII and transporting it to the site of primary hemostasis. Type 2N von Willebrand disease involves impaired binding of VWF to FVIII, resulting in decreased plasma levels of FVIII. OBJECTIVES: In these studies, we characterize the impact of three type 2N VWD variants (R763A, R854Q, R816W) on VWF secretion, FVIII stabilization, and thrombus formation in a murine model...
June 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28577390/feasibility-of-the-von-willebrand-disease-prevent-trial
#14
Margaret V Ragni, Nicoletta Machin, Andra H James, Craig D Seaman, Lynn M Malec, Craig M Kessler, Barbara A Konkle, Peter A Kouides, Anne T Neff, Claire S Philipp, Maria M Brooks
BACKGROUND: Despite treatment, women with von Willebrand disease (VWD) have lower von Willebrand factor (VWF) levels and greater blood loss at delivery than controls. Current weight-based dosing does not account for the ~1.5-fold increase in blood volume in pregnancy. METHODS: To evaluate the feasibility of a trial to prevent postpartum hemorrhage (PPH), we reviewed pre-pregnancy and 8th month VWF levels in women with VWD with and without PPH following vaginal delivery, assessed VWF concentrate use at delivery by U...
May 25, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28572165/long-term-impact-of-joint-bleeds-in-von-willebrand-disease-a-nested-case-control-study
#15
Karin P M van Galen, Piet de Kleijn, Wouter Foppen, Jeroen Eikenboom, Karina Meijer, Roger E G Schutgens, Kathelijn Fischer, Marjon H Cnossen, Joke de Meris, Karin Fijnvandraat, Johanna G van der Bom, Britta A P Laros-van Gorkom, Frank W G Leebeek, Evelien P Mauser-Bunschoten
Patients with severe von Willebrand Disease may develop arthropathy after joint bleeds. Limited information is available on its prevalence and severity. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. Von Willebrand Disease patients with and without verified joint bleeds were matched for age, gender and Factor VIII level or Von Willebrand Factor activity in a nested case-control study within the Willebrand in the Netherlands study. Assessments included the Hemophilia Joint Health Score (0-124), Pettersson score (0-13 per joint X-ray), Hemophilia Activity List score (0-100), joint pain (Visual Analog Scale 0-10) and Impact on Participation and Autonomy questionnaire (0-20)...
June 1, 2017: Haematologica
https://www.readbyqxmd.com/read/28559097/von-willebrand-disease-type-2-in-pregnancy-a-critical-clinical-association
#16
REVIEW
Anca A Simionescu, Natalia F Buinoiu, Nicoleta Berbec
Von Willebrand disease (vWD) is the most common inherited bleeding disorder. Its association with pregnancy is infrequent. Here we report three cases of vWD associated with pregnancy. The clinical features of this combination are very suggestive. However, difficulties are still encountered during the diagnosis process. The first case was diagnosed for the first time during the patient's second pregnancy despite several episodes of vaginal bleeding and suggestive clinical symptomatology. The second case was diagnosed during childhood and the patient was properly managed during this pregnancy...
May 18, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28544236/clinical-and-laboratory-phenotype-variability-in-type-2m-von-willebrand-disease
#17
A L Doruelo, S L Haberichter, P A Christopherson, L N Boggio, S Gupta, S R Lentz, A D Shapiro, R R Montgomery, V H Flood
Essentials The pathophysiology of type 2M von Willebrand disease (VWD) is poorly understood. Sequence variations in type 2M VWD subjects were characterized. A high degree of clinical and laboratory variability exists within type 2M VWD variants. Some type 2M variants may share features of type 2A VWD. SUMMARY: Background von Willebrand factor (VWF) is a multimeric coagulation factor that tethers platelets to injured subendothelium. Type 2M von Willebrand disease (VWD) is characterized by a qualitative defect in VWF with preserved multimer distribution...
May 20, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28536718/molecular-and-clinical-profile-of-vwd-in-a-large-cohort-of-chinese-population-application-of-next-generation-sequencing-and-cnvplex-%C3%A2-technique
#18
Qian Liang, Huanhuan Qin, Qiulan Ding, Xiaoling Xie, Runhui Wu, Hongli Wang, Yiqun Hu, Xuefeng Wang
Von Willebrand disease (VWD), the most common inherited bleeding disorder, is characterised by a variable bleeding tendency, heterogeneous laboratory phenotype and race specific distribution of mutations. The present study aimed to determine the correlation of genotype and phenotype in 200 Chinese individuals from 90 unrelated families with VWD. Next generation sequencing (NGS) of the whole coding VWF, copy number analysis of VWF by CNVplex® technique as well as a comprehensive phenotypic assessment were carried out in all index patients (IPs)...
May 24, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28533135/enhanced-local-disorder-in-a-clinically-elusive-von-willebrand-factor-provokes-high-affinity-platelet-clumping
#19
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 (VWF) 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, which quickly relapses back to normal platelet counts and deficient plasma VWF...
May 19, 2017: Journal of Molecular Biology
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
#20
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
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