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ADAMTS13

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https://www.readbyqxmd.com/read/29319374/sensitive-and-specific-assessment-of-recombinant-von-willebrand-factor-in-platelet-function-analyzer
#1
Isabell Pekrul, Thorsten Kragh, Peter L Turecek, Aaron R Novack, Helmut W Ott, Michael Spannagl
BACKGROUND: Recombinant von Willebrand factor (rVWF), which was licensed in the United States in 2015, has the multimeric distribution of freshly secreted VWF with ultralarge (UL) and high molecular weight multimers (HMWM) from endothelial cells and megakaryocytes since it has never been exposed to ADAMTS13 or any other proteolytic enzyme. Measurement of closure time (CT) using the platelet function analyzer-200 (PFA-200) is highly sensitive to the presence of UL VWF multimers in added VWF concentrates...
January 10, 2018: Platelets
https://www.readbyqxmd.com/read/29308213/case-series-of-3-patients-diagnosed-with-atypical-hemolytic-uremic-syndrome-successfully-treated-with-steroids-plasmapheresis-and-rituximab
#2
Jeffery M Patterson, Lauren Bolster, Loree Larratt
Rationale: Atypical hemolytic uremic syndrome, which has a high probability of chronic kidney disease, morbidity, and mortality, needs to be promptly recognized when patients present with microangiopathic hemolysis. Presenting Concerns of the Patient: Three patients present with laboratory parameters consistent with a thrombotic microangiopathy. With a suspected diagnosis of thrombotic thrombocytopenic purpura, steroids with plasmapheresis were initiated. Diagnoses: With ADAMTS13 levels reported normal, the suspected diagnoses were reevaluated...
2018: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29304532/platelet-count-adamts13-activity-von-willebrand-factor-level-and-survival-in-patients-with-colorectal-cancer-5-year-follow-up-study
#3
Nóra Garam, Éva Maláti, György Sinkovits, Tímea Gombos, Attila Szederjesi, Loránd Barabás, László Gráf, Judit Kocsis, Zoltán Prohászka
Distant metastasis is a major cause of colorectal cancer-related death, but the mechanism of tumour progression is not fully understood. There is growing evidence of an interaction between tumour cells and platelets which may influence tumour progression and metastasis formation. Quality and quantity of von Willebrand factor may regulate the interaction between tumour cells and platelets. Our aim was to measure the platelet count, von Willebrand factor antigen (VWF:Ag) levels and ADAMTS13 activity in a large (n = 232) cohort of colorectal cancer patients and to examine their relationships with the stage of the disease and 5-year survival without thrombotic complications using multivariable models...
January 2018: Thrombosis and Haemostasis
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/29296945/the-class-i-scavenger-receptor-cd163-promotes-internalization-of-adamts13-by-macrophages
#5
Fabian C Verbij, Nicoletta Sorvillo, Paul H P Kaijen, Johana Hrdinova, Ivan Peyron, Rob Fijnheer, Anja Ten Brinke, Alexander B Meijer, Floris P J van Alphen, Timo K van den Berg, Jonas J H Graversen, Soren K Moestrup, Jan Voorberg
Internalization of ADAMTS13 by macrophages may contribute to its clearance from the circulation. Here we investigated endocytic mechanisms that contribute to the uptake of ADAMTS13 by macrophages. Human monocyte-derived macrophages were used to monitor the uptake of fluorescently labeled recombinant ADAMTS13 by flow cytometry. Internalization of ADAMTS13 was blocked upon addition of the cell-permeable dynamin inhibitor dynasore. Partial blocking of ADAMTS13 uptake was observed by using mannan; however, uptake was not affected by an antibody that blocked binding to the macrophage mannose receptor CD206, which suggests that other endocytic receptors contribute to the internalization of ADAMTS13 by macrophages...
January 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296854/cyclosporine-or-steroids-as-an-adjunct-to-plasma-exchange-in-the-treatment-of-immune-mediated-thrombotic-thrombocytopenic-purpura
#6
Spero R Cataland, Peter J Kourlas, Shangbin Yang, Susan Geyer, Leslie Witkoff, Haiwa Wu, Camila Masias, James N George, Haifeng M Wu
Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296853/role-of-calcium-in-regulating-the-intra-and-extracellular-cleavage-of-von-willebrand-factor-by-the-protease-adamts13
#7
Shobhit Gogia, Anju Kelkar, Changjie Zhang, Kannayakanahalli M Dayananda, Sriram Neelamegham
von Willebrand factor (VWF) and the metalloprotease a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) are present both within endothelial cells (ECs) and in peripheral blood. Calcium concentrations are lower in intracellular compartments (80-400 μM) compared with the extracellular milieu (∼1.25 mM). Because low calcium favors VWF A2-domain proteolysis by ADAMTS13, the dependence of proteolysis rates on calcium was assayed both within ECs and in blood. Confocal microscopy studies demonstrate partial perinuclear colocalization of VWF with ADAMTS13 in human umbilical vein ECs (HUVECs)...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296808/severe-reduction-of-free-form-adamts13-unbound-to-von-willebrand-factor-in-plasma-of-patients-with-hellp-syndrome
#8
Yoko Yoshida, Masanori Matsumoto, Hideo Yagi, Ayami Isonishi, Kazuya Sakai, Masaki Hayakawa, Yuji Hori, Toshiyuki Sado, Hiroshi Kobayashi, Yoshihiro Fujimura
Severely decreased ADAMTS13 unbound to VWF may play a key role in the pathogenesis of HELLP syndrome.A qualitative ADAMTS13 assay may be important for diagnosing HELLP syndrome.
September 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296757/rituximab-prophylaxis-to-prevent-thrombotic-thrombocytopenic-purpura-relapse-outcome-and-evaluation-of-dosing-regimens
#9
John-Paul Westwood, Mari Thomas, Ferras Alwan, Vickie McDonald, Sylvia Benjamin, William A Lester, Gillian C Lowe, Tina Dutt, Quentin A Hill, Marie Scully
Acute antibody-mediated thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with high morbidity and mortality. Rituximab is highly effective as prophylaxis in patients at risk of acute TTP relapse, but the ideal dosing regimen is unknown. A multicenter retrospective cohort study evaluated outcomes of patients given rituximab prophylaxis to prevent TTP relapse. Rituximab was given in 76 episodes to 45 patients (34 women and 11 men). Four once-per-week infusions of standard- (375 mg/m2 [24 episodes]), reduced- (200 mg [19 episodes]), and intermediate- (500 mg [17 episodes]) dose rituximab were given; in the remaining 16 episodes, patients received 100 to 1000 mg rituximab in 1 to 5 doses...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296746/genetic-variants-in-adamts13-as-well-as-smoking-are-major-determinants-of-plasma-adamts13-levels
#10
Qianyi Ma, Paula M Jacobi, Brian T Emmer, Colin A Kretz, Ayse Bilge Ozel, Beth McGee, Chava Kimchi-Sarfaty, David Ginsburg, Jun Z Li, Karl C Desch
The metalloprotease ADAMTS13 cleaves von Willebrand factor (VWF) in circulating blood, limiting the size of VWF multimers and regulating VWF activity. Abnormal regulation of VWF contributes to bleeding and to thrombotic disorders. ADAMTS13 levels in plasma are highly variable among healthy individuals, although the heritability and the genetic determinants of this variation are unclear. We performed genome-wide association studies of plasma ADAMTS13 concentrations in 3244 individuals from 2 independent cohorts of healthy individuals...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296701/thrombotic-thrombocytopenic-purpura-diagnostic-criteria-clinical-features-and-long-term-outcomes-from-1995-through-2015
#11
Evaren E Page, Johanna A Kremer Hovinga, Deirdra R Terrell, Sara K Vesely, James N George
Our objective was to describe new observations from the Oklahoma Thrombotic Thrombocytopenic Purpura (TTP) Registry experience (November 1995 through December 2015) on the diagnosis of TTP along with patients' clinical features and their outcomes. Among 363 patients with an initial episode of clinically suspected TTP, the diagnosis of TTP was supported by both ADAMTS13 activity <10% and clinical features in 78 patients (21%). ADAMTS13 activity was measured in all 363 patients by 2 methods: fluorescence resonance energy transfer (FRET) and immunoblotting (IB)...
April 11, 2017: Blood Advances
https://www.readbyqxmd.com/read/29228282/proteolytic-inactivation-of-adamts13-by-plasmin-in-human-plasma-risk-of-thrombotic-thrombocytopenic-purpura
#12
Yongchol Shin, Haruki Miyake, Kenshi Togashi, Ryuichi Hiratsuka, Kana Endou-Ohnishi, Yasutada Imamura
Thrombotic thrombocytopenic purpura (TTP) is caused by inactivation of a von Willebrand factor (VWF)-cleaving enzyme, ADAMTS13, which leads to platelet-rich thrombi comprising unusually large VWF multimers. We have found that ADAMTS13 can bind to the inactivated form of plasmin. In addition, plasmin cleaves purified ADAMTS13 into several fragments and inactivates it. Hence, we hypothesized that activation of plasminogen to plasmin becomes a new-onset factor for TTP due to ADAMTS13 inactivation. Plasmin was added exogenously or activated from plasminogen by streprokinase addition in HP...
December 7, 2017: Journal of Biochemistry
https://www.readbyqxmd.com/read/29222940/an-open-conformation-of-adamts13-is-a-hallmark-of-acute-acquired-thrombotic-thrombocytopenic-purpura
#13
E Roose, A S Schelpe, B S Joly, M Peetermans, P Verhamme, J Voorberg, A Greinacher, H Deckmyn, S F De Meyer, P Coppo, A Veyradier, K Vanhoorelbeke
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is an autoimmune disorder characterized by absent ADAMTS13 activity and presence of anti-ADAMTS13 autoantibodies. Recently it was shown that ADAMTS13 adopts a folded or an open conformation. OBJECTIVES: As conformational changes in self-antigens play a role in the pathophysiology of different autoimmune diseases, we hypothesized that the conformation of ADAMTS13 changes during acute aTTP. METHODS: Antibodies recognizing cryptic epitopes in the spacer domain were generated...
December 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29222318/assessing-thrombocytopenia-in-the-intensive-care-unit-the-past-present-and-future
#14
REVIEW
Ryan Zarychanski, Donald S Houston
Thrombocytopenia is common among patients admitted to the intensive care unit (ICU). Multiple pathophysiological mechanisms may contribute, including thrombin-mediated platelet activation, dilution, hemophagocytosis, extracellular histones, ADAMTS13 deficiency, and complement activation. From the clinical perspective, the development of thrombocytopenia in the ICU usually indicates serious organ system derangement and physiologic decompensation rather than a primary hematologic disorder. Thrombocytopenia is associated with bleeding, transfusion, and adverse clinical outcomes including death, though few deaths are directly attributable to bleeding...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222317/thrombocytopenia-in-hospitalized-patients-approach-to-the-patient-with-thrombotic-microangiopathy
#15
REVIEW
Marie Scully
Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222249/thrombocytopenia-in-pregnancy
#16
REVIEW
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5% to 10% of women during pregnancy or in the immediate postpartum period. A low platelet count is often an incidental feature, but it might also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances made over the past 5 to 10 years in understanding and managing the more common causes of thrombocytopenia in pregnancy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29219891/thrombotic-thrombocytopenic-purpura-in-a-child-treated-for-acute-lymphoblastic-leukemia-case-report-and-review-of-literature
#17
Francesco De Leonardis, Roberta Koronica, Rosa Maria Daniele, Nicola Santoro
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by deficiency of von Willebrand factor-cleaving protease ADAMTS13. Large von Willebrand multimer formation and intravascular platelet aggregation affecting the arterioles and capillaries can result in death unless early treatment is administered. We report on the case of a child with TTP associated with a human herpes virus type-6 infection occurring during chemotherapy for acute lymphoblastic leukemia who was effectively treated by fresh frozen plasma infusions and antiviral therapy...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200971/vwf-adamts13-is-associated-with-on-aspirin-residual-platelet-reactivity-and-clinical-outcome-in-patients-with-stable-coronary-artery-disease
#18
Ellen M K Warlo, Alf-Åge R Pettersen, Harald Arnesen, Ingebjørg Seljeflot
Background: The mechanisms behind residual platelet reactivity (RPR) despite aspirin treatment are not established. It has been shown that coronary artery disease (CAD) patients with high on-aspirin RPR have elevated levels of von Willebrand factor (vWF). ADAMTS13 is a metalloprotease cleaving ultra large vWF multimers into less active fragments.Our aim was to investigate whether ADAMTS13 and vWF/ADAMTS13 ratio were associated with high RPR, and further with clinical endpoints after 2 years...
2017: Thrombosis Journal
https://www.readbyqxmd.com/read/29186156/a-common-mechanism-by-which-type-2a-von-willebrand-disease-mutations-enhance-adamts13-proteolysis-revealed-with-a-von-willebrand-factor-a2-domain-fret-construct
#19
Christopher J Lynch, Adam D Cawte, Carolyn M Millar, David Rueda, David A Lane
Rheological forces in the blood trigger the unfolding of von Willebrand factor (VWF) and its A2 domain, exposing the scissile bond for proteolysis by ADAMTS13. Under quiescent conditions, the scissile bond is hidden by the folded structure due to the stabilisation provided by the structural specialisations of the VWF A2 domain, a vicinal disulphide bond, a calcium binding site and a N1574-glycan.The reduced circulating high MW multimers of VWF in patients with type 2A von Willebrand disease (VWD) may be associated with mutations within the VWF A2 domain and this is attributed to enhanced ADAMTS13 proteolysis...
2017: PloS One
https://www.readbyqxmd.com/read/29178379/successful-treatment-of-thrombotic-microangiopathy-associated-to-dengue-infection-a-case-report-and-literature-review
#20
John Fredy Nieto-Ríos, María Fernanda Álvarez Barreneche, Sara Catalina Penagos, Diana Carolina Bello Márquez, Lina Maria Serna-Higuita, Isabel Cristina Ramírez Sánchez
Dengue infection has been associated with multiple renal complications, including glomerulonephritis, acute tubular necrosis, tubulointerstitial nephritis, and thrombotic microangiopathy (TMA); the last one being a rare complication of dengue, with only a few reported cases. TMA associated with dengue can be explained by an alteration in the activity of the enzyme ADAMTS13, leading to thrombotic thrombocytopenic purpura; or it can be secondary to direct or indirect endothelial injury by the virus, which leads to hemolytic uremic syndrome...
November 27, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
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