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acquired von willebrand

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https://www.readbyqxmd.com/read/29334169/impact-of-baseline-clinical-and-laboratory-features-on-the-risk-of-thrombosis-in-children-with-acute-lymphoblastic-leukemia-a-prospective-evaluation
#1
Uma H Athale, T Mizrahi, C Laverdière, T Nayiager, Y-L Delva, G Foster, L Thabane, M David, J-M Leclerc, A K C Chan
BACKGROUND: Children with acute lymphoblastic leukemia (ALL) have increased risk of thromboembolism (TE). However, the predictors of ALL-associated TE are as yet uncertain. OBJECTIVE: This exploratory, prospective cohort study evaluated the effects of clinical (age, gender, ALL risk group) and laboratory variables (hematological parameters, ABO blood group, inherited and acquired prothrombotic defects [PDs]) at diagnosis on the development of symptomatic TE (sTE) in children (aged 1 to ≤18) treated on the Dana-Farber Cancer Institute ALL 05-001 study...
January 15, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29331958/platelet-secretion-defects-and-acquired-von-willebrand-syndrome-in-patients-with-ventricular-assist-devices
#2
Ulrich Geisen, Kerstin Brehm, Georg Trummer, Michael Berchtold-Herz, Claudia Heilmann, Friedhelm Beyersdorf, Johannes Schelling, Axel Schlagenhauf, Barbara Zieger
BACKGROUND: The number of implanted ventricular assist devices (VADs) has increased significantly recently. Bleeding, the most frequent complication, cannot be solely attributed to anticoagulation therapy. Acquired von Willebrand syndrome (AVWS) caused by increased shear stress is frequent in VAD patients and can increase the bleeding risk. The HeartMate III (HM III) is a novel left VAD featuring potential improvements over the HeartMate II. METHODS AND RESULTS: In this study, we investigated the prevalence and onset of AVWS in 198 VAD patients...
January 13, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29321442/acquired-von-willebrand-syndrome-due-to-aortic-valve-stenosis-in-a-case-with-antiphospholipid-antibody
#3
Hiroaki Tanaka, Yurie Nagai, Chihiro Kuwabara, Ryo Shimizu, Akihide Umeki, Tetsufumi Yamamoto
Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29304523/insights-into-3d-structure-of-adamts13-a-stepping-stone-towards-novel-therapeutic-treatment-of-thrombotic-thrombocytopenic-purpura
#4
Bogac Ercig, Kanin Wichapong, Chris P M Reutelingsperger, Karen Vanhoorelbeke, Jan Voorberg, Gerry A F Nicolaes
ADAMTS13 (A D: isintegrin A: nd M: etalloprotease with a T: hromboS: pondin type-1 motif, member 13: ) and von Willebrand factor (VWF) can be considered as scale weights which control platelet adhesion during primary haemostasis. In a very uncommon condition designated thrombotic thrombocytopenic purpura (TTP), functional absence of ADAMTS13 tips the balance toward VWF-mediated platelet adhesion in the microcirculation. TTP is associated with a high mortality and arises from either a congenital or acquired autoimmune deficiency of the plasma enzyme ADAMTS13...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29274191/acquired-coagulopathy-in-patients-with-left-ventricular-assist-devices
#5
REVIEW
R Muslem, K Caliskan, F W G Leebeek
Chronic heart failure (HF) is a major emerging health care problem, associated with a high morbidity and mortality. Left ventricular assist devices (LVADs) have emerged as a successful treatment option for patients with end-stage HF. Despite its great benefit, the use of LVAD is associated with a high risk for complications. Bleeding, pump thrombosis, and thrombo-embolic events are frequently observed complications, with bleeding complications occurring in over a third of the patients. Although the design of the third generation LVAD has improved greatly, these hemostatic complications still occur...
December 23, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29240268/assessment-of-procoagulant-potential-in-patients-with-reactive-thrombocytosis-and-its-association-with-platelet-count
#6
Amihai Rottenstreich, Ela Shai, Geffen Kleinstern, Galia Spectre, David Varon, Yosef Kalish
OBJECTIVE: We aimed to determine hemostatic changes and characterize the procoagulant potential among reactive thrombocytosis (RT) patients. METHODS: Sixty RT patients (median platelet count 718X109 /L) and 20 healthy persons were tested for complete blood count, C-reactive protein, von Willebrand factor (VWF), factor VIII and fibrinogen, and thrombin generation. Platelet studies, including light transmission aggregometry and Cone and Plate(let) Analyzer, were also conducted...
December 14, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29237991/survival-of-heartmate-ii-patients-despite-cessation-of-anticoagulation%C3%A3-outcomes-and-hemostatic-analysis
#7
Rashad Zayat, Mohammad Amen Khattab, Oliver Grottke, Markus Honickel, Andreas Goetzenich, Ajay Moza, Christian Stoppe, Rüdiger Autschbach, Lachmandath Tewarie
BACKGROUND: In long-term left ventricular assist device (LVAD) therapy, recurrent bleeding events may justify cessation of anticoagulation therapy (AT). However, data about THE safety and risks of AT cessation in LVAD patients are scarce.Methods and Results:Between 2010 and 2015, 128 patients received a HeartMate II (HMII). Following recurrent bleeding events, we ceased vitamin K antagonist (VKA) therapy in 13 patients (10%) (no-VKA group). To characterize the hemostatic profile, we performed von Willebrand factor (vWF), platelet function (PF), and other hemostatic tests in all HMII patients...
December 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#8
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
https://www.readbyqxmd.com/read/29225984/acquired-von-willebrand-disease-associated-with-monoclonal-gammopathy-of-unknown-significance
#9
Sijan Basnet, Catherine Lin, Rashmi Dhital, Izza Mir, Elan Mohanty, Biswaraj Tharu, Sushil Ghimire, Dilli Ram Poudel
We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29202604/characterization-of-von-willebrand-factor-multimer-structure-in-patients-with-severe-aortic-stenosis
#10
Joerg Kellermair, Helmut W Ott, Michael Spannagl, Josef Tomasits, Juergen Kammler, Hermann Blessberger, Christian Reiter, Clemens Steinwender
Acquired von Willebrand syndrome (AVWS) associated with severe aortic stenosis (AS) has been frequently subclassified into a subtype 2A based on the deficiency of high-molecular-weight (HMW) multimers as it is seen in inherited von Willebrand disease (VWD) type 2A. However, the multimeric phenotype of VWD type 2A does not only include an HMW deficiency but also a decrease in intermediate-molecular-weight (IMW) multimers and an abnormal inner triplet band pattern. These additional characteristics have not been evaluated in AVWS associated with severe AS...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29188749/bleeding-during-veno-venous-ecmo-cannot-reliably-be-predicted-by-rotational-thrombelastometry-rotem%C3%A2
#11
Christina Hellmann, Axel Schmutz, Johannes Kalbhenn
INTRODUCTION: Veno-venous extracorporeal membrane oxygenation (vvECMO) used for respiratory support is associated with clinical bleeding in at least one third of patients. Mechanisms promoting bleeding, like acquired von Willebrand syndrome, cannot be identified by routine coagulation tests. This study was performed to evaluate rotational Thrombelastography (ROTEM™) for specific results predicting bleeding events during vvECMO. METHODS: Five hundred and thirty-four ROTEM™ analyses of 57 patients over 574 days have been evaluated...
November 1, 2017: Perfusion
https://www.readbyqxmd.com/read/29165741/emerging-roles-for-von-willebrand-factor-in-cancer-cell-biology
#12
Jamie M O'Sullivan, Roger J S Preston, Tracy Robson, James S O'Donnell
von Willebrand factor (VWF) is a complex multimeric plasma glycoprotein that plays critical roles in normal hemostasis. However, additional novel roles for VWF in modulating cancer cell biology, and in particular tumor metastasis, have recently been reported. Markedly elevated plasma VWF levels were associated with advanced tumor stage and metastatic disease. These observations have raised the question of whether VWF may be involved in regulating tumor progression. Interestingly, novel findings indicate that VWF is expressed by a variety of tumor cells of nonendothelial origin...
November 17, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29165737/management-of-hemostasis-for-pediatric-patients-on-ventricular-assist-devices
#13
Iki Adachi, Vadim Kostousov, Lisa Hensch, Martin A Chacon-Portillo, Jun Teruya
Ventricular-assist devices (VADs) have seen increased utilization in the pediatric population. Formerly, this therapeutic modality was limited to only the pulsatile VAD, EXCOR (Berlin Heart GmbH). However, the continuous flow VAD devices, HeartMate II (Abbott Inc.) and HeartWare (Medtronic Inc.), are now increasingly used in this population. Postoperatively, VAD patients are acutely anticoagulated using unfractionated heparin, often beginning 24 to 48 hours after VAD placement. Once the patient is stabilized and ready to transition to a lower acuity or outpatient setting, low-molecular-weight heparin or warfarin therapy may be instituted...
November 17, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29113538/acquired-von-willebrand-syndrome-in-an-infant-with-coarctation-of-the-aorta-and-williams-syndrome
#14
Clinton D Cochran, Rohit S Madani, Daniel Peltier, Steven Pipe, Sonal T Owens
An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29099367/dental-management-of-patients-with-inherited-bleeding-disorders-a-multidisciplinary-approach
#15
Hassan Abed, Abdalrahman Ainousa
Bleeding disorders can be inherited or acquired and demonstrate different levels of severity. Dentists may be called on to treat patients who have bleeding disorders such as hemophilia A and von Willebrand disease (vWD). Dental extraction in any patient with clotting factor defects can result in a delayed bleeding episode. Local hemostatic measures provide effective results in a majority of cases but are insufficient in patients with severe hemophilia A and vWD. Therefore, consultation with the patient's hematologist is required to ensure preoperative prophylactic coverage...
November 2017: General Dentistry
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#16
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/28987857/intracranial-hemorrhage-in-patients-with-durable-mechanical-circulatory-support-devices-institutional-review-and-proposed-treatment-algorithm
#17
Wyatt L Ramey, Robyn L Basken, Christina M Walter, Zain Khalpey, G Michael Lemole, Travis M Dumont
OBJECTIVE: Spontaneous intracranial hemorrhage (ICH) is frequently managed in neurosurgery. Patients with durable mechanical circulatory support devices, including total artificial hearts (TAHs) and left ventricular assist devices (LVADs) are often encountered in the setting of ICH. While durable mechanical circulatory support devices have improved survival and quality of life for patients with advanced heart failure, ICH is one of the most feared complications following LVAD and TAH implantation...
October 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28981198/amplified-endogenous-plasmin-activity-resolves-acute-thrombotic-thrombocytopenic-purpura-in-mice
#18
C Tersteeg, B S Joly, A Gils, R Lijnen, H Deckmyn, P J Declerck, B Plaimauer, P Coppo, A Veyradier, C Maas, S F De Meyer, K Vanhoorelbeke
Essentials Plasmin is able to proteolyse von Willebrand factor. It was unclear if plasmin influences acute thrombotic thrombocytopenic purpura (TTP). Plasmin levels are increased during acute TTP though suppressed via plasmin(ogen) inhibitors. Allowing amplified endogenous plasmin activity in mice results in resolution of TTP signs. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening pathology, caused by occlusive von Willebrand factor (VWF)-rich microthrombi that accumulate in the absence of ADAMTS-13...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28978901/aortic-valve-replacement-for-the-management-of-heyde-syndrome-a-case-report
#19
Akihiko Shibamoto, Hideto Kawaratani, Takuya Kubo, Norihisa Nishimura, Shinya Sato, Kenichiro Seki, Yasuhiko Sawada, Hiroaki Takaya, Yasushi Okura, Kousuke Takeda, Masakazu Uejima, Tadashi Namisaki, Kei Moriya, Akira Mitoro, Junichi Yamao, Hitoshi Yoshiji
Heyde syndrome describes the triad of aortic stenosis, acquired coagulopathy, and anemia due to bleeding from intestinal angiodysplasia. An 87-year-old man with iron deficiency anemia due to melena was admitted to our hospital. On examination, a systolic murmur was heard and echocardiography confirmed the presence of aortic stenosis. Esophagogastroduodenoscopy and colonoscopy were unremarkable. Capsule endoscopy and double balloon endoscopy revealed angiodysplasia throughout the small intestine. Laboratory investigations were significant for reduced plasma levels of high molecular weight von Willebrand factor multimers...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/28957820/risk-factors-of-gastrointestinal-bleeding-after-continuous-flow-left-ventricular-assist-device
#20
Ozlem Balcioglu, Hatice S Kemal, Serkan Ertugay, Pelin Ozturk, Yaprak Engin, Sanem Nalbantgil, Cagatay Engin, Tahir Yagdi, Mustafa Ozbaran
This study aimed to compare von Willebrand factor (vWF) levels, ristocetin cofactor levels, platelet counts, aortic valve movements, and right heart failure (RHF) as risk factors of gastrointestinal (GI) bleeding in patients with continuous flow left ventricular assist device (cf-LVAD). In a single centre, 90 patients (mean age 52.0 ± 10.5 years), of which 59 were male and 31 were female, had cf-LVAD implantation from October 2010 to November 2012. Seventy-six (84.4%) patients had HeartWare (Medtronic, Mounds View, MN) and 14 (15...
September 27, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
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