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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
Ashley L Doruelo, Sandra L Haberichter, Pamela A Christopherson, Lisa N Boggio, Sweta Gupta, Steven R Lentz, Amy D Shapiro, Robert R Montgomery, Veronica H Flood
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. OBJECTIVES: Through the Zimmerman Program for the Molecular and Clinical Biology for VWD, five VWF sequence variations were studied in subjects diagnosed with type 2M VWD. METHODS: Bleeding phenotype was assessed using the ISTH bleeding assessment tool...
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
#10
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
#11
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
#12
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/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#13
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/28492695/joint-assessment-in-von-willebrand-disease-validation-of-the-haemophilia-joint-health-score-and-haemophilia-activities-list
#14
Karin P M van Galen, Merel A Timmer, Piet de Kleijn, Kathelijn Fischer, Wouter Foppen, Roger E G Schutgens, Jeroen Eikenboom, Karina Meijer, Marjon H Cnossen, Karin Fijnvandraat, Johanna G van der Bom, Britta A P Laros-van Gorkom, Frank W G Leebeek, Eveline P Mauser-Bunschoten
Assessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28488237/perioperative-bleeding-and-thrombotic-risks-in-patients-with-von-willebrand-disease
#15
Nathaniel R Smilowitz, Navdeep Gupta, Yu Guo, Sripal Bangalore, Jeffrey S Berger
Von Willebrand disease (VWD) is an inherited bleeding disorder that often manifests clinically with hemorrhage after invasive procedures. We investigated the association between a diagnosis of VWD and bleeding and thrombotic outcomes following major non-cardiac surgery in a large national database from the United States. Patients age ≥45 years requiring major non-cardiac surgery were identified from Healthcare Cost and Utilization Project's National Inpatient Sample data. Von Willebrand disease, perioperative major adverse cardiovascular events (MACE), thrombotic events, and hemorrhage were defined by ICD9 diagnosis codes...
May 9, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28476066/angiodysplasia-in-von-willebrand-disease-understanding-the-clinical-and-basic-science
#16
Soundarya Selvam, Paula James
Severe and intractable gastrointestinal bleeding caused by angiodysplasia is a debilitating problem for up to 20% of patients with von Willebrand disease (VWD). Currently, the lack of an optimal treatment for this recurrent problem presents an ongoing challenge for many physicians in their management of affected patients. Over the past few years, studies have pointed to a regulatory role for the hemostatic protein, von Willebrand factor (VWF), in angiogenesis, providing a novel target for the modulation of vessel development...
May 5, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28453889/discrepant-platelet-and-plasma-von-willebrand-factor-in-von-willebrand-disease-patients-with-p-pro2808leufs-24
#17
M L Bowman, F G Pluthero, A Tuttle, L Casey, L Li, H Christensen, K S Robinson, D Lillicrap, W H A Kahr, P James
Essentials von Willebrand factor (VWF) is synthesized in endothelial cells and platelet precursors. Type 3 patients with Pro2808Leufs*24 have lower bleeding scores than other type 3s. The Pro2808Leufs*24 variant was examined in patient platelets and endothelial cells. Type 3s with this variant contain releaseable VWF, possibly reducing bleeding. SUMMARY: Background A novel variant, p.Pro2808Leufs*24, in the von Willebrand factor (VWF) gene was previously identified in the Canadian von Willebrand disease (VWD) patient population...
April 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28453185/generation-and-optimization-of-the-self-administered-pediatric-bleeding-questionnaire-and-its-validation-as-a-screening-tool-for-von-willebrand-disease
#18
Lara J Casey, Angie Tuttle, Julie Grabell, Wilma Hopman, Paul C Moorehead, Victor S Blanchette, John K Wu, MacGregor Steele, Robert J Klaassen, Mariana Silva, Margaret L Rand, Paula D James
OBJECTIVE: Our objective was to generate, optimize, and validate a self-administered pediatric bleeding questionnaire (Self-PBQ) as a screening tool for von Willebrand disease (VWD) in children referred to the hematology clinic for the first time. STUDY DESIGN: The Self-PBQ was generated by combining the validated expert-administered PBQ and the International Society on Thrombosis and Hemostasis (ISTH) bleeding assessment tool (BAT). Medical terminology was translated into lay language requiring a grade 4 reading level...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28447419/controversies-in-the-diagnosis-of-type-1-von-willebrand-disease
#19
REVIEW
M L Bowman, P D James
von Willebrand disease (VWD) is the most common bleeding disorder. Type 1 VWD represents the majority of cases and results from a partial quantitative deficiency of von Willebrand factor (VWF). The diagnosis of Type 1 VWD presents many challenges, despite there being three established diagnostic criteria: a personal history of mucocutaneous bleeding, a family history and low VWF levels. These criteria do not always coexist, and there is great overlap of clinical phenotypes and laboratory parameters between healthy individuals and those with VWD...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28436749/differential-diagnosis-of-neonatal-alloimmune-thrombocytopenia-type-2b-von-willebrand-disease
#20
Mathilde Penel-Page, Sandrine Meunier, Mathilde Fretigny, Sandra Le Quellec, Pierre Boisseau, Christine Vinciguerra, Catherine Ternisien, Lucia Rugeri
At birth, severe thrombocytopenia without context of infection should mainly suggest neonatal alloimmune thrombocytopenia (NAIT), especially in case of a platelet count below 20 GL(-1). We report two cases of severe neonatal thrombocytopenia, first suspected as being NAIT. Both had a platelet count below 20 GL(-1) with platelet clumps. The absence of alloantibodies and failure of platelet transfusion and intravenous immunoglobulins to improve the platelet count led to question the diagnosis and to evoke inherited bleeding disorders...
April 24, 2017: Platelets
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