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acquired von willebrand

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https://www.readbyqxmd.com/read/28187047/is-extracorporeal-co2-removal-really-safe-and-less-invasive-observation-of-blood-injury-and-coagulation-impairment-during-ecco2r
#1
Johannes Kalbhenn, Nadine Neuffer, Barbara Zieger, Axel Schmutz
Extracorporeal CO2-Removal (ECCO2R) is promoted with attributes like "safe" and "less invasive" compared to (high-flow) veno-venous ECMO-Systems. With our experience in coagulation disorders during ECMO-therapy with this observational study we for the first time prospectively evaluate hemolysis and coagulation disorders during ECCO2R. Eight consecutive patients with predominant hypercapnic respiratory failure were treated with the Hemolung® Respiratory Assist System (RAS) (Alung-Technologies, Pittsburg, USA)...
February 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28181217/hypercoagulability-and-migraine
#2
Gretchen E Tietjen, Stuart A Collins
BACKGROUND: A growing body of literature suggests that migraineurs, particularly those with aura, have an increased risk for ischemic stroke, but not via enhanced atherosclerosis. The theory that micro-emboli induced ischemia provokes cortical spreading depression (ie, symptomatic aura) in migraineurs but transient ischemic attacks in others highlights a potential role for hypercoagulability as a link between migraine (with aura) and stroke. AIM: Our objective is to summarize the literature evaluating the association of migraine with various acquired or inheritable thrombophilic states, including those related to elevated estrogen levels, endothelial activation and dysfunction, antiphospholipid antibodies (aPL), deficiency of coagulation inhibitors, and presence of certain genetic polymorphisms...
February 9, 2017: Headache
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#3
REVIEW
Paul 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. In this context, the introduction of rituximab has probably 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, or even as frontline therapy, with high response rates...
December 30, 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28103720/crohn-s-disease-and-acquired-von-willebrand-syndrome-a-rare-dangerous-affair
#4
Federico Pasin, Sophie Testa, Pietro Capone, Federico Buffoli, Antonio Cuzzoli, Giovanni Paolo Coppeta, Roberto Grassia
No abstract text is available yet for this article.
January 20, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28088607/transcatheter-aortic-valve-implantation-leads-to-a-restoration-of-von-willebrand-factor-vwf-abnormalities-in-patients-with-severe-aortic-stenosis-incidence-and-relevance-of-clinical-and-subclinical-vwf-dysfunction-in-patients-undergoing-transfemoral-tavi
#5
Alexander Sedaghat, Hannah Kulka, Jan-Malte Sinning, Nora Falkenberg, Julia Driesen, Barbara Preisler, Christoph Hammerstingl, Georg Nickenig, Bernd Pötzsch, Johannes Oldenburg, Hans-Jörg Hertfelder, Nikos Werner
BACKGROUND: In this study, we sought to analyze the incidence and relevance of von Willebrand factor (VWF) abnormalities in patients undergoing transcatheter aortic valve implantation (TAVI), especially on perioperative bleeding. Furthermore, we hypothesized that, similar to aortic valve surgery, TAVI results in a restoration of VWF abnormalities. METHODS AND RESULTS: We performed a prospective analysis of periinterventional VWF parameters in 74 patients (80±7years, female in 37...
January 7, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28060126/pediatric-acquired-von-willebrand-syndrome-in-cardiopulmonary-disorders-do-laboratory-abnormalities-predict-bleeding-risk
#6
Saman K Hashmi, Mireya P Velasquez, Donald L Yee, Shiu-Ki Hui, Donald Mahoney, Lakshmi V Srivaths
There are conflicting reports on whether or not laboratory abnormalities in pediatric acquired von Willebrand syndrome (AVWS) predict bleeding manifestations in patients with cardiopulmonary disorders (CPD). We retrospectively reviewed charts of patients with AVWS and CPD (n=16) seen at Texas Children's Hospital from 2003 to 2012. The most common CPD were valve stenoses, ventricular septal defects, and pulmonary hypertension. All patients had loss of high molecular weight multimers. Fifteen (94%) patients presented with bleeding symptoms, with menorrhagia and epistaxis being the most common...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28035064/functional-assessment-of-von-willebrand-factor-expression-by-cancer-cells-of-non-endothelial-origin
#7
Anahita Mojiri, Konstantin Stoletov, Maria Areli Lorenzana Carrillo, Lian Willetts, Saket Jain, Roseline Godbout, Paul Jurasz, Consolato M Sergi, David D Eisenstat, John D Lewis, Nadia Jahroudi
Von Willebrand factor (VWF) is a highly adhesive procoagulant molecule that mediates platelet adhesion to endothelial and subendothelial surfaces. Normally it is expressed exclusively in endothelial cells (ECs) and megakaryocytes. However, a few studies have reported VWF detection in cancer cells of non-endothelial origin, including osteosarcoma. A role for VWF in cancer metastasis has long been postulated but evidence supporting both pro- and anti-metastatic roles for VWF has been presented. We hypothesized that the role of VWF in cancer metastasis is influenced by its cellular origin and that cancer cell acquisition of VWF expression may contribute to enhanced metastatic potential...
December 27, 2016: Oncotarget
https://www.readbyqxmd.com/read/28028990/acquired-von-willebrand-syndrome
#8
REVIEW
Andrzej Mital
Acquired von Willebrand syndrome is a rare hemorrhagic diathesis, with clinical symptoms similar to those associated with the inherited form von Willebrand disease. This syndrome is characterized by a lack of previous bleeding symptoms, negative familial history, and occurrence in a relatively older age. Most commonly, acquired von Willebrand syndrome develops in the course of other conditions, such as lymphoproliferative, myeloproliferative, cardiovascular and autoimmune disorders; additionally, it can be associated with some non-hematological malignancies and use of certain prescription drugs...
November 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28017358/differentiation-of-mesenchymal-stem-cells-from-human-amniotic-fluid-to-vascular-endothelial-cells
#9
Waleephan Tancharoen, Sirinda Aungsuchawan, Peraphan Pothacharoen, Runchana Markmee, Suteera Narakornsak, Junjira Kieodee, Nonglak Boonma, Witoon Tasuya
Endothelial dysfunction is a principle feature of vascular-related disease. Endothelial cells have been acquired for the purposes of the restoration of damaged tissue in therapeutic angiogenesis. However, their use is limited by expansion capacity and the small amount of cells that are obtained. Human amniotic fluid mesenchymal stem cells (hAF-MSCs) are considered an important source for vascular tissue engineering. In this study, hAF-MSCs were characterized and then induced in order to differentiate into the endothelial-like cells...
March 2017: Acta Histochemica
https://www.readbyqxmd.com/read/28011677/n-acetylcysteine-in-preclinical-mouse-and-baboon-models-of-thrombotic-thrombocytopenic-purpura
#10
Claudia Tersteeg, Jan Roodt, Walter J Van Rensburg, Charlotte Dekimpe, Nele Vandeputte, Inge Pareyn, Aline Vandenbulcke, Barbara Plaimauer, Seb Lamprecht, Hans Deckmyn, José A Lopez, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder diagnosed by thrombocytopenia and hemolytic anemia, associated with a deficiency in von Willebrand factor (VWF) cleaving protease ADAMTS13. Current treatment is based on plasma infusion for congenital TTP, or plasma exchange, often in combination with immunosuppressive agents, for acquired TTP. These treatment methods are however not always effective and therefore new treatment methods are highly necessary. N-acetylcysteine (NAC), an FDA-approved anti-mucolytic agent, could be a possible new treatment strategy for TTP as it was demonstrated to reduce disulfide bonds in VWF, thereby decreasing VWF multimer size and hence its prothrombotic potential...
December 23, 2016: Blood
https://www.readbyqxmd.com/read/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#11
Ayalew Tefferi, Tiziano Barbui
DISEASE OVERVIEW: Polycythemia Vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. DIAGNOSIS: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27922890/tolerability-and-biological-effects-of-long-acting-octreotide-in-patients-with-continuous-flow-left-ventricular-assist-devices
#12
Rajiv Malhotra, Keyur B Shah, Raveen Chawla, Sammy Pedram, Melissa C Smallfield, Anna G Priday, Christine T DeWilde, Donald F Brophy
Patients with implanted continuous, non-pulsatile, left ventricular assist devices (LVADs) have increased occurrence of gastrointestinal bleeding (GIB). While the pathophysiology is multifactorial, there are few treatments beyond supportive care. Octreotide acetate is a somatostatin analog that reduces GIB in various patient populations. However, there are sparse case-series that suggest octreotide acetate may reduce GIB in LVAD patients. This 10 patient, 28-week Phase I study evaluated the safety and tolerability of octreotide acetate long-acting release (LAR) 20 mg depot injection every four weeks until week 16 following LVAD placement...
December 2, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27919526/factors-related-to-the-development-of-acquired-von-willebrand-syndrome-in-patients-with-essential-thrombocythemia-and-polycythemia-vera
#13
A Rottenstreich, G Kleinstern, S Krichevsky, D Varon, D Lavie, Y Kalish
OBJECTIVE: We characterized acquired von Willebrand syndrome (AVWS) among essential thrombocythemia (ET) and polycythemia vera (PV) patients. METHODS: A review of patients with ET or PV evaluated for AVWS. RESULTS: Of 116 patients with ET, 64 (55%) developed AVWS; of 57 with PV, 28 (49%) developed AVWS. Median platelet counts of ET and PV patients who developed AVWS were 920×10(9)/L and 679×10(9)/L, respectively (P=0.01). Of patients who developed AVWS, 69...
December 2, 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27851358/1723-acquired-von-willebrand-disease-in-essential-thrombocythemia
#14
Kathryn Kaye Berlin, Patrick Foy
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27834763/acquired-von-willebrand-syndrome-an-under-recognized-cause-of-major-bleeding-in-the-cardiac-intensive-care-unit
#15
Melissa B Jones, Karthik Ramakrishnan, Fahad A Alfares, Kendal M Endicott, Gary Oldenburg, John T Berger, Venkat Shankar, Dilip S Nath, Yaser A Diab
BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27806384/diagnostic-challenges-in-acquired-von-willebrand-syndrome-a-complex-case-of-prostate-carcinoma-associated-acquired-von-willebrand-syndrome
#16
Paul-Emile Claus, Inge Van Haute, Eline Verhoye, Dries Deeren, Els Moreau
No abstract text is available yet for this article.
February 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/27762046/immunochip-analysis-identifies-novel-susceptibility-loci-in-the-human-leukocyte-antigen-region-for-acquired-thrombotic-thrombocytopenic-purpura
#17
I Mancini, I Ricaño-Ponce, E Pappalardo, A Cairo, M M Gorski, G Casoli, B Ferrari, M Alberti, D Mikovic, M Noris, C Wijmenga, F Peyvandi
Essentials Genetic predisposition to acquired thrombotic thrombocytopenic purpura (aTTP) is mainly unknown. Genetic risk factors for aTTP were studied by Immunochip analysis and replication study. Human leukocyte antigen (HLA) variant rs6903608 conferred a 2.5-fold higher risk of developing aTTP. rs6903608 and HLA-DQB1*05:03 may explain most of the HLA association signal in aTTP. Click to hear Dr Cataland's presentation on acquired thrombotic thrombocytopenic purpura SUMMARY: Background Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor-cleaving protease ADAMTS-13...
December 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27676646/evidence-of-both-von-willebrand-factor-deposition-and-factor-v-deposition-onto-al-amyloid-as-the-cause-of-a-severe-bleeding-diathesis
#18
Jonathan S Harrison, Shellaine R Frazier, Dianne D McConnell, Senan John Yasar, Nataliya Melnyk, Gratian Salaru
Acquired coagulopathies are common; uncommonly, adsorption of coagulation factors from the circulation into the tissues by pathologic amyloid exceeds the body's ability to produce factor and results in acquired factor deficiency. When amyloidosis does cause a coagulopathy, it is most often acquired factor X deficiency, but there are rare reports of amyloidosis being associated with other acquired factor deficiencies. We investigated a case of a severe bleeding diathesis, the cause of which was combined acquired factor V deficiency and concomitant acquired von Willebrand syndrome...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27648200/effects-of-factor-xiii-deficiency-on-thromboelastography-thromboelastography-with-calcium-and-streptokinase-addition-is-more-sensitive-than-solubility-tests
#19
M Martinuzzo, L Barrera, D Altuna, F Tisi Baña, J Bieti, Q Amigo, M D'Adamo, M S López, J Oyhamburu, J C Otaso
BACKGROUND: Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to compare them to the clot solubility studies results in samples of a 1-year-old girl with homozygous or double heterozygous FXIII deficiency...
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27622788/utility-of-the-von-willebrand-factor-collagen-binding-assay-in-the-diagnosis-of-von-willebrand-disease
#20
Emmanuel J Favaloro
von Willebrand Disease (VWD) is the most common inherited bleeding disorder and also arises as an acquired defect (AVWS). VWD and AVWS are due to quantitative deficiencies and/or qualitative defects in von Willebrand factor (VWF), an adhesive plasma protein with multiple activities. Diagnosis of VWD is problematic, being subject to overdiagnosis, underdiagnosis, and misdiagnosis. This is largely due to limitations in current test procedures and an over-reliance on these imperfect test systems for clinical diagnosis...
January 2017: American Journal of Hematology
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