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acquired von willebrand

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https://www.readbyqxmd.com/read/29760727/expanded-cd133-cells-from-human-umbilical-cord-blood-improved-heart-function-in-rats-after-severe-myocardial-infarction
#1
Alejandro Correa, Gabriel Salles Ottoboni, Alexandra Cristina Senegaglia, Luiz Guilherme Achcar Capriglione, Nelson Itiro Miyague, Lidiane Maria Boldrini Leite, Valderez Ravaglio Jamur, Carmen Lúcia Kuniyoshi Rebelatto, Márcia Olandoski, Paulo Roberto Slud Brofman
Pharmacological approaches are partially effective in limiting infarct size. Cell therapies using a cell population enriched with endothelial progenitor cells (EPCs) CD133+ have opened new perspectives for the treatment of ischemic areas after infarction. This preclinical study evaluated the effect of intramyocardial transplantation of purified or expanded human umbilical cord blood-derived CD133+ cells on the recovery of rats following acute myocardial infarction (AMI). Histology studies, electrocardiogram, and fluorescence in situ hybridization (FISH) were used to evaluate heart recovery...
2018: Stem Cells International
https://www.readbyqxmd.com/read/29747831/arterial-pulsatility-and-circulating-von-willebrand-factor-in-patients-on-mechanical-circulatory-support
#2
Flavien Vincent, Antoine Rauch, Valentin Loobuyck, Emmanuel Robin, Christoph Nix, André Vincentelli, David M Smadja, Pascal Leprince, Julien Amour, Gilles Lemesle, Hugues Spillemaeker, Nicolas Debry, Christian Latremouille, Piet Jansen, Antoine Capel, Mouhamed Moussa, Natacha Rousse, Guillaume Schurtz, Cédric Delhaye, Camille Paris, Emmanuelle Jeanpierre, Annabelle Dupont, Delphine Corseaux, Mickaël Rosa, Yoann Sottejeau, Svenja Barth, Claudia Mourran, Valérie Gomane, Augustin Coisne, Marjorie Richardson, Claudine Caron, Cristian Preda, Alexandre Ung, Alain Carpentier, Thomas Hubert, Cécile Denis, Bart Staels, Peter J Lenting, Eric Van Belle, Sophie Susen
BACKGROUND: The main risk factor for bleeding in patients with continuous-flow mechanical circulatory support (CF-MCS) is the acquired von Willebrand factor (VWF) defect related to the high shear-stress forces developed by these devices. Although a higher bleeding rate has been reported in CF-MCS recipients who had reduced pulsatility, the relation between pulsatility and the VWF defect has never been studied. OBJECTIVES: The purpose of this study was to investigate the relation between pulsatility and VWF under CF-MCS...
May 15, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29746258/gi-bleeding-during-cf-lvad-support-state-of-the-field
#3
Rachna Kataria, Ulrich P Jorde
Continuous-flow left ventricular assist devices (CF-LVADs) are increasingly used for the management of advanced heart failure refractory to optimal medical therapy. Despite the encouraging outcomes with CF-LVADs, gastrointestinal bleeding (GIB) continues to be a rather concerning complication resulting in increased rates of readmission and increased morbidity. The exact pathophysiology of CF-LVAD associated GIB remains poorly understood and this lack of knowledge limits our ability to control this morbid complication...
May 9, 2018: Cardiology in Review
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#4
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29741685/association-between-the-severity-of-acquired-von-willebrand-syndrome-and-gastrointestinal-bleeding-after-continuous-flow-left-ventricular-assist-device-implantation
#5
Ko Sakatsume, Kenki Saito, Masatoshi Akiyama, Konosuke Sasaki, Satoshi Kawatsu, Goro Takahashi, Osamu Adachi, Shunsuke Kawamoto, Hisanori Horiuchi, Yoshikatsu Saiki
OBJECTIVES: Acquired von Willebrand syndrome, characterized by the reduction in von Willebrand factor (vWF) large multimers, has recently been considered as one of the causes of gastrointestinal bleeding (GIB). It remains unclear whether its haematological severity is linked with susceptibility to bleeding because the definition of the haematological severity of acquired von Willebrand syndrome has not been precisely determined. This study sought to establish a quantitative methodology to assess the haematological severity of acquired von Willebrand syndrome and to define the threshold for occurrence of GIB in patients implanted with left ventricular assist devices (LVADs)...
May 8, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29741250/how-i-investigate-for-bleeding-disorders
#6
REVIEW
C P M Hayward
INTRODUCTION: Laboratory investigations for bleeding disorders are warranted when an individual has a personal and/or family history of bleeding, and/or laboratory findings that suggest the possibility of an inherited or acquired bleeding disorder. METHODS: This review summarizes author's experience with ordering and reporting on diagnostic investigations for common and rare bleeding disorders, with consideration of recent articles on diagnosing bleeding disorders...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#7
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29715362/characterizing-the-heartmate-ii-left-ventricular-assist-device-outflow-using-particle-image-velocimetry
#8
Grant W Rowlands, Bryan C Good, Steven Deutsch, Keefe B Manning
Ventricular assist devices (VADs) are implanted in patients with a diseased ventricle to maintain peripheral perfusion as a bridge-to-transplant or as destination therapy. However, some patients with continuous flow VADs (e.g., HeartMate II (HMII)) have experienced gastrointestinal (GI) bleeding, in part caused by the proteolytic cleavage or mechanical destruction of von Willebrand factor (vWF), a clotting glycoprotein. in vitro studies were performed to measure the flow located within the HMII outlet cannula under both steady and physiological conditions using particle image velocimetry (PIV)...
July 1, 2018: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/29651351/acquired-von-willebrand-disease-associated-with-mantle-cell-lymphoma
#9
Dominique Maas, Britta Laros-van Gorkom, Sanne Gianotten, Marjan Cruijsen, Waander van Heerde, Marten Nijziel
We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma. A 61-year-old male suffered from recurrent bleeding symptoms since a few months. Initially, physical examination was normal. von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31 IU/ml and 0.43 IU/ml, resp.). Ristocetin cofactor activity (VWF:RCo) was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml. VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29650295/acquired-von-willebrand-syndrome-and-impaired-platelet-function-during-venovenous-extracorporeal-membrane-oxygenation-rapid-onset-and-fast-recovery
#10
Johannes Kalbhenn, Axel Schlagenhauf, Simone Rosenfelder, Axel Schmutz, Barbara Zieger
BACKGROUND: Bleeding contributes to the high mortality of venovenous extracorporeal membrane oxygenation (vvECMO). The development of acquired von Willebrand syndrome (AVWS) has been identified as relevant pathology during ECMO. This study was performed to determine the onset of AVWS after implantation and the recovery of von Willebrand factor (VWF) parameters after explantation of ECMO in a large cohort of patients. METHODS: VWF parameters of 59 patients treated with vvECMO at a university ECMO center were obtained before ECMO implantation, during therapy, and after explantation...
March 17, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29619625/effects-of-plasma-glycosyltransferase-on-the-abo-h-blood-group-antigens-of-human-von-willebrand-factor
#11
Taiki Kano, Kazunao Kondo, Jiharu Hamako, Fumio Matsushita, Kazuya Sakai, Taei Matsui
Von Willebrand factor (VWF) is one of the plasma protein carrying ABO(H) blood group antigens, but the combining process of these antigens is not clear. In the present study, we examined whether plasma glycosyltransferase affects the blood group antigens on VWF. VWF expressing H-antigen (H-VWF) from blood group O and bovine serum albumin conjugated with H-antigen (H-BSA) were incubated with recombinant α1-3-N-acetylgalactosaminyltransferase (rA-transferase) and A-plasma with or without an additional UDP-GalNAc...
April 4, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29608457/can-the-plasmaderived-factor-viii-still-play-a-role-in-the-treatment-of-acquired-hemophilia-a-at-the-time-of-new-drugs
#12
Samantha Pasca, Vincenzo De Angelis, Marta Milan, Ezio Zanon
: Bypassing agents are the first-line therapy in the treatment of acquired hemophilia A (AHA), but not the only one. Other options as recombinant porcine factor VIII or plasmaderived concentrates (pdFVIII) are available to clinicians. Aim of this study was to evaluate whether the pdFVIII can still play a role in the treatment of AHA, and which patients could benefit from this therapy. All patients with AHA, presenting severe cardiovascular comorbidities, and treated with pdFVIII with or without von Willebrand factor (vWF), referred to two different hospitals, were initially considered...
March 31, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29575615/spectrum-of-hemostatic-disorders-in-indian-females-presenting-with-bleeding-manifestations
#13
A Gupta, P Mishra, H P Pati, S Tyagi, M Mahapatra, T Seth, R Saxena
INTRODUCTION: Hemostatic disorders are often missed in women with bleeding particularly menorrhagia. Preexisting hemostatic disorders are now known as common risk factor for postpartum hemorrhage and prolonged bleeding in puerperium. Females with bleeding complaints constitute an important population referred to hematology clinic. Hence, we aim to evaluate the type and frequency of hemostatic disorders among females presenting with bleeding in a tertiary care hospital and a basic hemostatic laboratory...
March 25, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29573126/the-influence-of-thyroid-function-on-the-coagulation-system-and-its-clinical-consequences
#14
REVIEW
L P B Elbers, E Fliers, S C Cannegieter
Several studies indicate that low plasma levels of thyroid hormone shift the hemostatic system towards a hypocoagulable and hyperfibrinolytic state, whereas high levels of thyroid hormone lead to more coagulation and less fibrinolysis. Low levels of thyroid hormone thereby seem to lead to an increased bleeding risk, whereas high levels, by contrast, increase the risk of venous thromboembolism. Hypothyroidism leads to a higher incidence of acquired von Willebrand's syndrome and with increasing levels of free thyroxine, levels of fibrinogen, factor VIII and von Willebrand factor, amongst others, increase gradually, to the extent that they may lead to symptomatic venous thromboembolism in patients with hyperthyroidism...
April 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29568223/acquired-von-willebrand-syndrome-complicating-nephrotic-syndrome-a-case-of-a-patient-with-membranous-nephropathy
#15
Taro Sugase, Tetsu Akimoto, Takaaki Kimura, Takashi Yagisawa, Eiji Kusano, Daisuke Nagata
Acquired von Willebrand syndrome (AVWS) is a rare clinical entity presenting with heterogeneous hemorrhagic manifestations, although some subsets of patients with AVWS may be asymptomatic until they are exposed to major trauma, an invasive procedure, or surgery. We herein report one such case in a 73-year-old male patient with nephrotic syndrome with a prolonged active partial thromboplastin time. We initially did not deal with this distinct abnormal clotting profile seriously, but persistent bleeding after a retroperitoneoscopic-assisted renal biopsy that allowed us to ascribe his nephrotic syndrome to membranous nephropathy fortuitously led to the discovery of concurrent AVWS...
2018: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29567779/mass-spectrometry-assisted-identification-of-adamts13-derived-peptides-presented-on-hla-dr-and-hla-dq
#16
Johana Hrdinová, Fabian C Verbij, Paul H P Kaijen, Robin B Hartholt, Floris van Alphen, Neubury Lardy, Anja Ten Brinke, Karen Vanhoorelbeke, Pooja J Hindocha, Anne S De Groot, Alexander B Meijer, Jan Voorberg, Ivan Peyron
Formation of microthrombi is a hallmark of acquired thrombotic thrombocytopenic purpura. These microthrombi originate from insufficient processing of ultra large von Willebrand factor multimers by ADAMTS13 due to the development of anti-ADAMTS13 autoantibodies. Several studies have identified the major histocompatibility complex class II alleles HLA-DRB1*11, HLA-DQB1*03 and HLA-DQB1*02:02 as risk factors for acquired thrombotic thrombocytopenic purpura development. Previous research in our department indicated that ADAMTS13 CUB2 domain-derived peptides FINVAPHAR and LIRDTHSLR are presented on HLA-DRB1*11 and HLA-DRB1*03, respectively...
March 22, 2018: Haematologica
https://www.readbyqxmd.com/read/29564686/ofatumumab-for-acute-treatment-and-prophylaxis-of-a-patient-with-multiple-relapses-of-acquired-thrombotic-thrombocytopenic-purpura
#17
Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
March 21, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29512410/experimental-measurement-and-numerical-modelling-of-dye-washout-for-investigation-of-blood-residence-time-in-ventricular-assist-devices
#18
Alessandra Molteni, Zubair Ph Masri, Kenny Wq Low, Haitham N Yousef, Johann Sienz, Katharine H Fraser
Ventricular assist devices have become the standard therapy for end-stage heart failure. However, their use is still associated with severe adverse events related to the damage done to the blood by fluid dynamic stresses. This damage relates to both the stress magnitude and the length of time the blood is exposed to that stress. We created a dye washout technique which combines experimental and numerical approaches to measure the washout times of ventricular assist devices. The technique was used to investigate washout characteristics of three commercially available and clinically used ventricular assist devices: the CentriMag, HVAD and HeartMate II...
April 2018: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/29443370/beneficial-effects-of-high-density-lipoproteins-on-acquired-von-willebrand-syndrome-in-aortic-valve-stenosis
#19
C Gebhard, F Maafi, B E Stähli, A Bonnefoy, C E Gebhard, W Nachar, A Benjamim de Oliveira Moraes, M Mecteau, T Mihalache-Avram, V Lavoie, A E Kernaleguen, Y Shi, D Busseuil, M Chabot-Blanchet, L P Perrault, D Rhainds, E Rhéaume, J C Tardif
BACKGROUND:  Infusions of apolipoprotein A-I (apoA-I), the major protein component of high-density lipoproteins (HDL), result in aortic valve stenosis (AVS) regression in experimental models. Severe AVS can be complicated by acquired von Willebrand syndrome, a haemorrhagic disorder associated with loss of high-molecular-weight von Willebrand factor (vWF) multimers (HMWM), the latter being a consequence of increased shear stress and enhanced vWF-cleaving protease (ADAMTS-13) activity...
February 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29406356/gastrointestinal-bleeding-in-left-ventricular-assist-device-octreotide-and-other-treatment-modalities
#20
Tara L Molina, Jill Krisl, Kevin Donahue, Sara Varnado
Left ventricular assist devices (LVADs) offer a therapeutic strategy for patients with end-stage heart failure. Increased device utilization has also increased the incidence of device-related complications including gastrointestinal bleeding (GIB). Multiple mechanisms have been proposed in the pathophysiology of continuous-flow LVAD-associated GIB including physiologic changes associated with high shear and nonpulsatile flow such as gastrointestinal arteriovenous malformations and acquired von Willebrand syndrome...
February 5, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
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