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acquired von willebrand

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https://www.readbyqxmd.com/read/27922890/tolerability-and-biological-effects-of-long-acting-octreotide-in-patients-with-continuous-flow-left-ventricular-assist-devices
#1
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
#2
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
#3
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
#4
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
#5
Paul-Emile Claus, Inge Van Haute, Eline Verhoye, Dries Deeren, Els Moreau
No abstract text is available yet for this article.
November 2, 2016: 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
#6
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...
October 20, 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
#7
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
#8
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
#9
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...
September 13, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27616321/an-11-year-experience-of-acquired-von-willebrand-syndrome-in-children-diagnosed-with-wilms-tumour-in-a-tertiary-referral-centre
#10
Emma Fosbury, Elwira Szychot, Olga Slater, Mary Mathias, Keith Sibson
Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology...
September 12, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27601054/mechanical-circulatory-support-is-associated-with-loss-of-platelet-receptors-glycoprotein-ib%C3%AE-and-glycoprotein-vi
#11
P Lukito, A Wong, J Jing, J F Arthur, S F Marasco, D A Murphy, P J Bergin, J A Shaw, M Collecutt, R K Andrews, E E Gardiner, A K Davis
: Essentials Relationship of acquired von Willebrand disease (VWD) and platelet dysfunction is explored. Patients with ventricular assist devices and on extracorporeal membrane oxygenation are investigated. Acquired VWD and platelet receptor shedding is demonstrated in the majority of patients. Loss of platelet adhesion receptors glycoprotein (GP) Ibα and GPVI may increase bleeding risk. SUMMARY: Background Ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO) are associated with bleeding that is not fully explained by anticoagulant or antiplatelet use...
September 7, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27587498/pediatric-acquired-von-willebrand-disease-with-berlin-heart-excor-ventricular-assist-device-support
#12
REVIEW
Nathan Gossai, Nicholas M Brown, Rebecca Ameduri, Nicole D Zantek, James St Louis, Marie E Steiner
BACKGROUND: The balance of hemostasis and anticoagulation is a concern for patients dependent upon ventricular assist devices (VADs). Bleeding is a common complication with both short- and long-term use of these devices. A better understanding of the risk factors and etiologies of bleeding associated with these devices is needed and could improve the overall results. We sought to determine the relationship of mechanical circulatory assist device use with acquired von Willebrand disease (avWD) in children...
September 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27587252/-acquired-von-willebrand-syndrome-in-three-patients-and-literature-review
#13
H Y Ouyang, Z J Yu, J Yin, X J Zhao, Z Y Wang, W Zhang, Z N Ma, J Su, X Bai, C G Ruan
OBJECTIVE: To deepen the understanding of acquired von Willebrand syndrome (AVWS). METHODS: The clinical data of 3 patients were analyzed and related literature were reviewed. RESULTS: ① Case 1, a 70- year- old male, diagnosed as Waldenstrom macroglobulinemia and AVWS, was presented with spontaneous epitaxis and bruising. The VWF∶Ag level was 16%. Treatment was initiated with VWF concentrates. Two cycles of chemotherapy with Bortezomib, thalidomide and Dexamethasone were followed...
August 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27581227/von-willebrand-factor-and-prekallikrein-in-plasma-are-associated-with-thrombus-volume-in-abdominal-aortic-aneurysms
#14
Qasam M Ghulam, Kim K Bredahl, Jørgen B Gram, Lars Lönn, Jens P Goetze, Henrik H Sillesen, Jonas P Eiberg
OBJECTIVES: Disruption of the endothelial lining may be one of the events linking intraluminal thrombus and abdominal aortic aneurysm growth. In the present study, we examined whether von Willebrand factor activity in plasma, contact proteins of blood coagulation, and inflammatory biomarkers may be associated with intraluminal thrombus volume in search of a biochemical marker of endothelial damage and thrombus size. DESIGN: Prospective study, correlating potential endothelial biomarkers and intraluminal thrombus volume acquired by computed tomography angiography...
August 2016: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/27576087/use-and-future-investigations-of-recombinant-and-plasma-derived-coagulation-and-anticoagulant-products-in-the-neonate
#15
REVIEW
Rowena C Punzalan, Jerome L Gottschall
Although congenital bleeding disorders can manifest in the newborn period, the most common causes of bleeding and thrombosis in neonates are acquired conditions. Factor concentrates are used for specific diagnoses (hemophilia with inhibitors, specific factor deficiency, von Willebrand disease) and approved indications, and increasingly for off-label indications (bleeding in surgery cardiopulmonary bypass, extracorporeal membrane oxygenation). We will review the approved indications for factor products in the neonate and discuss the evidence and rationale for off-label use of factor products in management of bleeding and thrombosis in the neonate...
October 2016: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/27556142/inhaled-desmopressin-for-refractory-gastrointestinal-bleeding-in-a-patient-with-a-heartmate-ii-%C3%A2-left-ventricular-assist-device
#16
Ian B Hollis, Sheh-Li Chen, Patricia P Chang, Jason N Katz
Patients with a durable, continuous-flow left ventricular assist device (LVAD) are commonly prescribed the combination of an oral anticoagulant and an oral antiplatelet agent as prophylaxis against device thrombosis and systemic embolic events. Current guidelines recommend warfarin with an INR goal of 2-3 and concomitant aspirin 81-325mg daily for patients with a HeartMate II ® LVAD (HM II). Unfortunately, gastrointestinal bleeding (GIB) is very common in these patients due to multiple factors including the development of arteriovenous malformations (AVMs) and acquired von Willebrand syndrome (AvWS)...
August 22, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27505249/determinants-of-acquired-activated-protein-c-resistance-and-d-dimer-in-breast-cancer
#17
Mari Tinholt, Per Morten Sandset, Marie-Christine Mowinckel, Øystein Garred, Kristine Kleivi Sahlberg, Vessela N Kristensen, Anne-Lise Børresen-Dale, Anne Flem Jacobsen, Grethe Skretting, Nina Iversen
BACKGROUND: We have previously reported acquired activated protein C (APC) resistance and elevated plasma D-dimer levels in breast cancer patients. Here, we aimed to identify phenotypic and genetic determinants that contribute to the acquired APC resistance and increased D-dimer levels in breast cancer. Healthy controls served as reference. We also addressed whether higher APC resistance or D-dimer levels could be potential markers of clinicopathological breast cancer characteristics...
September 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27485105/effects-of-heartware-ventricular-assist-device-on-the-von-willebrand-factor-results-of-an-academic-belgian-center
#18
Fatemeh Esmaeilzadeh, Aurélien Wauters, Walter Wijns, Jean-François Argacha, Philippe van de Borne
BACKGROUND: Left Ventricular Assist Device (LVAD) is a promising therapy for patients with advanced heart failure (HF), but bleeding complications remain an important issue. Previous series show that acquired von Willebrand syndrome was present in up to 100 % of first generation LVAD recipients. We report the effects of new generation LVADs on vW factor (vWF) metabolism and activity in our center. METHODS: Fifteen LVAD recipients (HeartWare®, Framingham, MA, USA) were compared to 12 HF patients, matched for age and body mass index...
2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27484220/identification-and-analysis-of-copine-bonzai-proteins-among-evolutionarily-diverse-plant-species
#19
Baohong Zou, Xuexue Hong, Yuan Ding, Xiang Wang, He Liu, Jian Hua
Copines are evolutionarily conserved calcium-dependent membrane-binding proteins with potentially critical biological functions. In plants, the function of these proteins has not been analyzed except for in Arabidopsis thaliana where they play critical roles in development and disease resistance. To facilitate functional studies of copine proteins in crop plants, genome-wide identification, curation, and phylogeny analysis of copines in 16 selected plant species were conducted. All the identified 32 plant copines have conserved features of the two C2 domains (C2A and C2B) and the von Willebrand factor A (vWA) domain...
August 2016: Genome Génome / Conseil National de Recherches Canada
https://www.readbyqxmd.com/read/27481874/severe-aortic-valve-stenosis-sustained-cure-of-acquired-von-willebrand-syndrome-after-surgical-valve-replacement
#20
Rolf Dario Frank, Regina Lanzmich, Philipp K Haager, Ulrich Budde
Aortic valve stenosis (AVS) is the most common valve disease in adults. Severe forms are associated with acquired von Willebrand syndrome (aVWS) with loss of the largest von Willebrand factor (VWF) multimers. Diagnostic gold standard is the VWF multimer analysis. Valve replacement rapidly restores the VWF structure. Uncertainty exists if this effect is permanent and how functional VWF assays perform compared with multimer analysis. We studied 21 consecutive patients with severe AVS before and 6 to 18 months after valve surgery and compared them with 14 controls without valve disease referred for coronary angiography...
August 1, 2016: Clinical and Applied Thrombosis/hemostasis
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