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https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#1
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
June 20, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28641568/an-expanding-role-of-biomarkers-in-pulmonary-arterial-hypertension
#2
Mustafa Yildiz, Alparslan Sahin, Michael Behnes, İbrahim Akin
BACKGROUND: Pulmonary arterial hypertension (PAH), is a chronic disease which may cause or result from mulitple cardiopulmonary disorders. The disease has complex pathophysiological mechanisms and involves many systematic, cellular and molecular changes. Therefore, it is crutial to find out underlying mechanisms and detect biomarkers to achieve early and proper diagnosis, evaluate of disease severity, for follow-up and monitor response to treatment. Many biomarker for PAH have been investigated but yet no such biomarker has been found specific and easily accessible to use for the patients...
June 14, 2017: Current Pharmaceutical Biotechnology
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
#3
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
#4
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/28634421/the-role-of-von-willebrand-factor-in-vascular-inflammation-from-pathogenesis-to-targeted-therapy
#5
REVIEW
Felice Gragnano, Simona Sperlongano, Enrica Golia, Francesco Natale, Renatomaria Bianchi, Mario Crisci, Fabio Fimiani, Ivana Pariggiano, Vincenzo Diana, Andreina Carbone, Arturo Cesaro, Claudia Concilio, Giuseppe Limongelli, Mariagiovanna Russo, Paolo Calabrò
Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28618874/von-willebrand-factor-multimeric-structure-and-functional-activity-in-patients-with-atrial-fibrillation-with-and-without-oral-anticoagulation
#6
Sandra Lopez-Castaneda, Ignacio Valencia-Hernández, Carlos Arean, Daniel Godínez-Hernández, Martha Eva Viveros-Sandoval
von Willebrand factor (vWF) is a multimeric glycoprotein present in blood plasma. It is synthesized in megakaryocytes and endothelial cells, secreted into circulation in the form of high-molecular-weight multimers (HMWMs), and cleaved into shorter, less active multimers by ADAMTS13. It is essential for platelet adhesion and aggregation. Previous studies have investigated the relationship between vWF levels and thromboembolic events with little regard to vWF multimeric structure. Patients with atrial fibrillation (AF) exhibit higher plasma vWF and lower ADAMTS13 levels...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28612396/diagnosis-of-inherited-bleeding-disorders-in-the-genomic-era
#7
REVIEW
Suthesh Sivapalaratnam, Janine Collins, Keith Gomez
Inherited bleeding disorders affect between 1 in 1000 individuals for the most common disorder, von Willebrand Disease, to only 8 reported cases worldwide of alpha-2-antiplasmin deficiency. Those with an identifiable abnormality can be divided into disorders of coagulation factors (87%), platelet count and function (8%) and the fibrinolytic system (3%). Of the patients registered in the UK with a bleeding disorder, the remaining 2% are unclassifiable. In addition to bleeding symptoms, patients with an inherited bleeding disorder can manifest other abnormalities, making an accurate and complete diagnosis that reflects the underlying molecular pathology important...
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28610577/coronary-angiogenic-effect-of-long-term-administration-of-nigella-sativa
#8
Lubna I Al Asoom
BACKGROUND: Coronary angiogenesis is one of the preferable adaptive responses of aerobic training. Previous studies found inotropic and hypertrophic cardiac effects for long-term administration of Nigella sativa (NS), but no studies have explored its coronary angiogenic effect. The present study compared the effect of long-term NS- administration and exercise training on the induction of coronary angiogenesis. METHOD: Fifteen adult male Wistar rats were divided into three groups: control, NS-fed, and exercise-trained (Ex)...
June 13, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28609796/successful-use-of-a-highly-purified-plasma-von-willebrand-factor-concentrate-containing-little-fviii-for-the-long-term-prophylaxis-of-severe-type-3-von-willebrand-s-disease
#9
Tomas Simurda, Miroslava Dobrotova, Ingrid Skornova, Juraj Sokol, Peter Kubisz, Jan Stasko
No abstract text is available yet for this article.
June 13, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28599697/paraneoplastic-cushing-syndrome-due-to-wilm-s-tumor
#10
Mahwish Faizan, Jaida Manzoor, Muhammad Saleem, Saadia Anwar, Qaiser Mehmood, Ambreen Hameed, Agha Shabbir Ali
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28598970/seroprevalence-and-clinical-characteristics-of-viral-hepatitis-in-transfusion-dependent-thalassemia-and-hemophilia-patients
#11
Tyng-Yuan Jang, Pei-Chin Lin, Ching-I Huang, Yu-Mei Liao, Ming-Lun Yeh, Yu-Sheng Zeng, Po-Cheng Liang, Wan-Yi Hsu, Shih-Pien Tsai, Zu-Yau Lin, Shinn-Cherng Chen, Jee-Fu Huang, Chia-Yen Dai, Chung-Feng Huang, Shyh-Shin Chiou, Wan-Long Chuang, Ming-Lung Yu
BACKGROUND/AIMS: Transfusion dependent subjects are at a great risk of viral hepatitis infection. We aimed to evaluate the prevalence and factors associated with hepatitis B virus (HBV) and hepatitis C virus (HCV) infection among transfusion-dependent patients in Taiwan. METHODS: A total of 140 patients (67 thalassemic patients, 70 hemophilic patients, two patients with hereditary spherocytosis and one patient with von Willebrand disease) were prospectively enrolled to evaluate the prevalence and factors associated with viral hepatitis and spontaneous HCV clearance...
2017: PloS One
https://www.readbyqxmd.com/read/28597906/platelet-receptors-as-therapeutic-targets-past-present-and-future
#12
Janina Jamasbi, Keng Ayabe, Shinya Goto, Bernhard Nieswandt, Karlheinz Peter, Wolfgang Siess
Anti-platelet drugs reduce arterial thrombosis after plaque rupture and erosion, prevent stent thrombosis and are used to prevent and treat myocardial infarction and ischaemic stroke. Some of them may also be helpful in treating less frequent diseases such as thrombotic thrombocytopenic purpura. The present concise review aims to cover current and future developments of anti-platelet drugs interfering with the interaction of von Willebrand factor (VWF) with glycoprotein (GP) Ibα, and directed against GPVI, GPIIb/IIIa (integrin αIIbβ3), the thrombin receptor PAR-1, and the ADP receptor P2Y12...
June 8, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28591212/reduced-adamts13-levels-in-patients-with-acute-and-chronic-cerebrovascular-disease
#13
Frederik Denorme, Peter Kraft, Inge Pareyn, Christiane Drechsler, Hans Deckmyn, Karen Vanhoorelbeke, Christoph Kleinschnitz, Simon F De Meyer
Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is known about a correlation with ADAMTS13. Therefore, the aim of this study was to compare plasma levels of ADAMTS13 between 85 healthy volunteers (HV), 104 patients with acute ischemic stroke and 112 patients with a chronic cerebrovascular disease (CCD). In this case-control study, plasma ADAMTS13 antigen levels were measured by ELISA and plasma VWF levels, measured previously, were next used to calculate VWF:ADAMTS13 ratios...
2017: PloS One
https://www.readbyqxmd.com/read/28587726/occult-cancer-detection-in-patients-with-hemostatic-disorder-and-venous-thromboembolism
#14
Holleh Husseinzadeh, Marc Carrier
There are physiologic ties between Von Willebrand Factor (VWF) and circulating tumor cells. VWF appears to play a role in tumor biology, but it is unclear whether cancer behavior differs in Von Willebrand Disease. In patients presenting with venous thromboembolism (VTE), occult cancer is frequently considered as an underlying cause. The prevalence of occult cancer after provoked VTE is low (3%); therefore, cancer screening in these patients is not routinely recommended. In those with unprovoked VTE, occult cancer is more prevalent, estimated between 4 and 10%...
June 1, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28586149/comparative-analysis-of-von-willebrand-factor-profiles-after-implantation-of-left-ventricular-assist-device-and-total-artificial-heart
#15
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/28581694/abnormal-von-willebrand-factor-secretion-factor-viii-stabilization-and-thrombus-dynamics-in-type-2n-von-willebrand-disease-mice
#16
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/28580812/platelet-reactivity-in-stable-cardiovascular-patients-with-chronic-kidney-disease
#17
Thomas A Mavrakanas, Ahsan Alam, Jean-Luc Reny, Pierre Fontana
The study aimed to evaluate antiplatelet drug responsiveness in stable outpatients with cardiovascular disease and chronic kidney disease (CKD) and examine whether impaired antiplatelet drug responsiveness is associated with worse clinical outcomes in this population. Stable cardiovascular patients (n = 771) were enrolled at least one month after an acute ischemic atherothrombotic event. Antiplatelet drug responsiveness was assessed with specific assays (serum TxA2 for aspirin, the VASP assay for clopidogrel) and other aggregation-based assays using different agonists...
June 5, 2017: Platelets
https://www.readbyqxmd.com/read/28577390/feasibility-of-the-von-willebrand-disease-prevent-trial
#18
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/28576889/blood-coagulation-system-in-patients-with-chronic-kidney-disease-a-prospective-observational-study
#19
Meng-Jie Huang, Ri-Bao Wei, Yang Wang, Ting-Yu Su, Ping Di, Qing-Ping Li, Xi Yang, Ping Li, Xiang-Mei Chen
OBJECTIVES: Thromboembolic events are the major factor affecting the prognosis of patients with chronic kidney disease (CKD). Haemostatic alterations are possible causes of these complications, but their roles remain poorly characterised. In the prospective observational study, we investigated the entire coagulation process in patients with CKD to elucidate the mechanisms of their high thromboembolic risk. METHODS: A total of 95 patients with CKD and 20 healthy controls who met the inclusion criteria were consecutively recruited from September 2015 to March 2016...
June 1, 2017: BMJ Open
https://www.readbyqxmd.com/read/28572286/gut-microbiota-regulate-hepatic-von-willebrand-factor-synthesis-and-arterial-thrombus-formation-via-toll-like-receptor-2
#20
Sven Jäckel, Klytamnistra Kiouptsi, Maren Lillich, Tim Hendrikx, Avinash Khandagale, Bettina Kollar, Nives Hörmann, Cora Reiss, Saravanan Subramaniam, Eivor Wilms, Katharina Ebner, Marie-Luise von Brühl, Philipp Rausch, John F Baines, Sandra Haberichter, Bernhard Lämmle, Christoph J Binder, Kerstin Jurk, Zaverio M Ruggeri, Steffen Massberg, Ulrich Walter, Wolfram Ruf, Christoph Reinhardt
The symbiotic gut microbiota plays pivotal roles in host physiology and the development of cardiovascular diseases, but microbiota-triggered pattern recognition signaling mechanisms impacting thrombosis are poorly defined. Here, we show that germ-free and Toll-like receptor (Tlr) 2-deficient mice have reduced thrombus growth following carotid artery injury relative to conventionally raised controls. Germ-free Tlr2(-/-) and wild-type mice were indistinguishable, but colonization with microbiota restored a significant difference in thrombus growth between genotypes...
June 1, 2017: Blood
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