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https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#1
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#2
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28319521/warfarin-and-aspirin-versus-warfarin-alone-for-prevention-of-embolic-events-in-patients-with-a-heartmate-ii%C3%A2-left-ventricular-assist-device
#3
Joseph S Van Tuyl, Ian B Hollis, Khalid A Alburikan, Richard Tran, Brian P Murray, Jo E Rodgers, Jason N Katz, Brett C Sheridan
Acquired von Willebrand disease increases bleeding risk in patients implanted with a continuous-flow left ventricular assist device. Lower aspirin doses decrease the risk of bleeding without an increased risk of embolic events. No published studies in the U.S. have compared the incidence of bleeding and thrombotic events between antithrombotic regimens with and without aspirin. A single-center, retrospective analysis was conducted of adult patients implanted with a HM II. Patients received warfarin and aspirin 81 mg daily or warfarin alone...
March 17, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#4
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28236847/is-von-willebrand-disease-linked-to-cholesteatoma-aetiology
#5
 Reis Rego, M Santos, M Coutinho, T Feliciano, C Almeida E Sousa
Cholesteatoma is a common clinical picture seen by otolaryngologists. The disease is characterised by an abnormal grow "of skin in the wrong place". Specifically, in acquired cholesteatoma, the main causative issue is associated with ventilatory deficits within the middle ear and results in chronic infection. Molecular science has associated the presence of specific molecules with its development, and moreover recent research suggests that deregulated angiogenesis is a crucial process in the development of cholesteatoma and its recurrence...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#6
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...
February 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28103720/crohn-s-disease-and-acquired-von-willebrand-syndrome-a-rare-dangerous-affair
#7
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/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/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#10
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/27851358/1723-acquired-von-willebrand-disease-in-essential-thrombocythemia
#11
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
#12
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/27762046/immunochip-analysis-identifies-novel-susceptibility-loci-in-the-human-leukocyte-antigen-region-for-acquired-thrombotic-thrombocytopenic-purpura
#13
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/27622788/utility-of-the-von-willebrand-factor-collagen-binding-assay-in-the-diagnosis-of-von-willebrand-disease
#14
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
https://www.readbyqxmd.com/read/27587498/pediatric-acquired-von-willebrand-disease-with-berlin-heart-excor-ventricular-assist-device-support
#15
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
#16
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/27576087/use-and-future-investigations-of-recombinant-and-plasma-derived-coagulation-and-anticoagulant-products-in-the-neonate
#17
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/27484220/identification-and-analysis-of-copine-bonzai-proteins-among-evolutionarily-diverse-plant-species
#18
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/27479501/generation-of-anti-murine-adamts13-antibodies-and-their-application-in-a-mouse-model-for-acquired-thrombotic-thrombocytopenic-purpura
#19
Louis Deforche, Claudia Tersteeg, Elien Roose, Aline Vandenbulcke, Nele Vandeputte, Inge Pareyn, Elien De Cock, Hanspeter Rottensteiner, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate development and validation of new therapies for this disease. Therefore, a large panel (n = 19) of novel anti-mouse ADAMTS13 (mADAMTS13) monoclonal antibodies (mAbs) of mouse origin was generated. Inhibitory anti-mADAMTS13 mAbs were identified using the FRETS-VWF73 assay. Four mAbs strongly inhibited mADAMTS13 activity in vitro (∼68-90% inhibition)...
2016: PloS One
https://www.readbyqxmd.com/read/27443161/acute-compartment-syndrome-as-the-initial-manifestation-of-chronic-phase-chronic-myeloid-leukemia-a-case-report-and-review-of-the-literature
#20
Yoshikazu Nagase, Shuji Ueda, Hitomi Matsunaga, Aya Yoshioka, Yoshiyuki Okada, Tomohisa Machida, Keiichi Nakata, Fuka Mima, Risato Takeda, Daisuke Hayashi, Sadaharu Iio, Kohei Okita, Hiroyuki Narahara, Yuichi Yasunaga, Yoshiaki Inui, Sumio Kawata
BACKGROUND: Acute compartment syndrome is an orthopedic emergency requiring urgent fasciotomy to prevent irreversible damage. In hematological malignancies, acute compartment syndrome caused by severe soft tissue bleeding is extremely rare. We present a patient with chronic-phase chronic myeloid leukemia who had acute compartment syndrome caused by severe soft tissue bleeding in her right forearm. CASE PRESENTATION: A 72-year-old Japanese woman was referred to our hospital with swelling and pain of her right forearm without a previous history of trauma...
July 21, 2016: Journal of Medical Case Reports
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