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ADAMTS-13

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https://www.readbyqxmd.com/read/29618156/adamts-13-activity-predicts-outcome-in-acute-ischaemic-stroke-patients-undergoing-endovascular-treatment
#1
Ramona Schuppner, Meike Dirks, Gerrit M Grosse, Matthias Böckmann, Friedrich Goetz, Thomas Pasedag, Stefanie M Bode-Böger, Jens Martens-Lobenhoffer, Ulrich Budde, Heinrich Lanfermann, Ralf Lichtinghagen, Karin Weissenborn, Hans Worthmann
BACKGROUND:  Endovascular treatment improves outcome in patients with acute ischaemic stroke due to large vessel occlusion in general. But outcome in some of these patients is jeopardized by recanalization failure or bleeding. OBJECTIVES:  This study aimed to determine a possible association of mediators of inflammation and haemostasis (C-reactive protein, interleukin-6, matrix metalloproteinase-9, monocyte chemoattractant protein-1, asymmetric dimethylarginine [ADMA], symmetric dimethylarginine, von Willebrand factor and a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13 [ADAMTS-13]) with the post-intervention grade of reperfusion, complications and clinical outcome in patients who underwent endovascular treatment of ischaemic stroke...
April 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29594148/c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-two-important-manifestations-of-complement-system-dysfunction
#2
Ravneet Bajwa, John A DePalma, Taimoor Khan, Anmol Cheema, Sheila A Kalathil, Mohammad A Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif
The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29567368/efficacy-of-eculizumab-in-severe-adamts13-deficient-thrombotic-thrombocytopenic-purpura-ttp-refractory-to-standard-therapies
#3
Ernesto Vigna, Annamaria Petrungaro, Anna Perri, Dario Terzi, Anna Grazia Recchia, Francesco Mendicino, Antonella La Russa, Sabrina Bossio, Laura De Stefano, Francesco Zinno, Renzo Bonofiglio, Fortunato Morabito, Massimo Gentile
Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathic hemolytic anemia (MAHA) defined by mechanical hemolytic anemia, severe thrombocytopenia, and systemic visceral ischemia due to systemic platelet-rich microthrombi. Forty percent of patients with autoimmune TTP experience one or multiple relapses. Patients with refractory TTP are currently managed by corticosteroids, twice-daily PEX, and the anti-CD20 monoclonal antibody rituximab. Herein, we report two cases of severe TTP, refractory to those standard agents...
March 15, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29443370/beneficial-effects-of-high-density-lipoproteins-on-acquired-von-willebrand-syndrome-in-aortic-valve-stenosis
#4
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. Although antithrombotic actions of HDL have been described, its effects on ADAMTS-13 and vWF in AVS are unknown...
February 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29435466/opana-er-oxymorphone-induced-thrombotic-microangiopathy-an-atypical-presentation-in-a-patient-with-hepatitis-c
#5
Hassan Mehmood, Muzammil Khan, Asghar Marwat, Medha Joshi, Varun Malhotra
Oxymorphone is a semisynthetic extended release opiate used to treat moderate to severe chronic pain. The Food and Drug Administration approved the oral form of oxymorphone available as Opana and Opana ER (extended release) since 2006. The Food and Drug Administration and the Centers for Disease Control and Prevention issued warning against intravenous use of Opana ER. We are presenting a case report of a 37-year-old female with a history of active intravenous drug abuse who presented to our hospital with acute kidney injury...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29344548/recombinant-adamts-13-in-thrombotic-thrombocytopenic-purpura
#6
EDITORIAL
Marie Scully, Christopher Hibbard, Bruce Ewenstein
No abstract text is available yet for this article.
November 2017: Oncoscience
https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#7
REVIEW
M Levi, M Scully, M Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor (VWF) is the most prominent. The multimeric size of VWF is an important determinant of a more intense platelet-vessel wall interaction, and is regulated by the VWF-cleaving protease ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultralarge VWF multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura, a thrombotic microangiopathy characterized by thrombocytopenia, non-immune hemolysis, and organ dysfunction...
April 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29329518/the-use-of-eculizumab-in-gemcitabine-induced-thrombotic-microangiopathy
#8
Vinod Krishnappa, Mohit Gupta, Haikoo Shah, Abhijit Das, Natthavat Tanphaichitr, Robert Novak, Rupesh Raina
BACKGROUND: Thrombotic microangiopathy (TMA) secondary to gemcitabine therapy (GiTMA) is a very rare pathology that carries a poor prognosis, with nearly half of the cases progressing to end stage renal disease. GiTMA is most commonly associated with adenocarcinomas, most notably pancreatic cancers. The mainstay of management is withdrawal of the offending drug and supportive care. Plasmapheresis has a limited role and hemodialysis may help in the management of fluid overload secondary to renal failure...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29135310/adamts-1-4-12-and-13-levels-in-maternal-blood-cord-blood-and-placenta-in-preeclampsia
#9
Müberra Namlı Kalem, Ziya Kalem, Tuncay Yüce, Feride Soylemez
The aim of this study is to compare ADAMTS (A Disintegrin and Metalloprotease Domains with Thrombospondins motifs) 1, 4, 12, and 13 levels in maternal and cord blood and placental tissue between preeclampsia and uncomplicated pregnancies. The enzyme-linked immunosorbent assay (ELISA) results showed that ADAMTS 1, 4, 12, and 13 levels in the maternal and cord blood were lower in the preeclampsia group than in the control group. Based on the immunohistochemistry (IHC) results, ADAMTS 1, 4, and 12 levels in placental tissues were higher in the preeclampsia group...
February 2018: Hypertension in Pregnancy
https://www.readbyqxmd.com/read/29130325/the-plasma-levels-of-adamts-13-von-willebrand-factor-vwfpp-and-fibrin-related-markers-in-patients-with-systemic-sclerosis-having-thrombosis
#10
Koji Habe, Hideo Wada, Ayaka Higashiyama, Tomoko Akeda, Kenshiro Tsuda, Ryoko Mori, Masato Kakeda, Takeshi Matsumoto, Kohshi Ohishi, Keiichi Yamanaka, Naoyuki Katayama, Hitoshi Mizutani
This study aimed to examine the hemostatic abnormalities in patients with systemic sclerosis (SSc) and the relationship between these abnormalities and thrombotic events (THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp), d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. The relationship between their levels and organ involvement, including THEs and interstitial lung disease (ILD), was evaluated...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29122768/recombinant-adamts-13-goodbye-allergic-reactions
#11
COMMENT
Ravi Sarode
No abstract text is available yet for this article.
November 9, 2017: Blood
https://www.readbyqxmd.com/read/29064619/external-validation-of-the-plasmic-score-a-clinical-prediction-tool-for-thrombotic-thrombocytopenic-purpura-diagnosis-and-treatment
#12
A Li, P R Khalighi, Q Wu, D A Garcia
Essentials Severe ADAMTS-13 deficiency is key to thrombotic thrombocytopenic purpura (TTP) diagnosis. PLASMIC score predicts ADAMTS-13 deficiency in suspected TTP with high discrimination. PLASMIC score is more generalizable with fewer missing data than alternative clinical scores. PLASMIC score identifies a subgroup of patients lacking significant response to plasma exchange. SUMMARY: Background The PLASMIC score was recently published to distinguish patients with severe ADAMTS-13 deficiency from those without for early identification of thrombotic thrombocytopenia purpura (TTP)...
January 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28981194/elevated-preoperative-von-willebrand-factor-is-associated-with-perioperative-thrombosis-in-infants-and-neonates-with-congenital-heart-disease
#13
R Hunt, C M Hoffman, S Emani, C C Trenor, S M Emani, D Faraoni, C Kimchi-Sarfaty, J C Ibla
Essentials Perioperative thrombosis is a major cause of morbidity and mortality in congenital heart disease. Neonates and infants undergoing repair of congenital heart lesions were prospectively followed. Elevated von Willebrand factor (VWF) to ADAMTS-13 activity ratios typified the postoperative period. Thrombosis was associated with preoperative VWF activity and cryoprecipitate transfusion SUMMARY: Background The surgical repair of congenital heart malformations is frequently complicated by perioperative thrombosis of unclear etiology...
December 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28942075/clinical-and-in-vitro-evidence-that-subclinical-hemolysis-contributes-to-lvad-thrombosis
#14
Carlo R Bartoli, David Zhang, Jooeun Kang, Samson Hennessy-Strahs, David Restle, Jessica Howard, Gretchen Redline, Christian Bermudez, Pavan Atluri, Michael A Acker
BACKGROUND: Recent data suggest that hemolysis contributes to left ventricular assist device (LVAD) thrombosis, but the mechanism is unknown. In a clinical study, we measured plasma free hemoglobin (pfHgb) and the incidence of LVAD thrombosis. In an in vitro study, we examined biophysical relationships between shear stress, pfHgb and von Willebrand factor (vWF) metabolism toward understanding mechanisms of LVAD thrombosis. METHODS: In the clinical study, blood samples were obtained from continuous-flow LVAD patients (n = 30)...
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28927148/association-of-abo-blood-groups-with-von-willebrand-factor-factor-viii-and-adamts-13-in-patients-with-lung-cancer
#15
Xia Liu, Xiaogang Chen, Jiezuan Yang, Renyong Guo
Coagulative and fibrinolytic disorders appear to be associated with the development of lung cancer. The aim of the present study was to determine plasma levels of von Willebrand factor (VWF) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13 (ADAMTS-13), and factor VIII (FVIII) activity, in association with O and non-O blood groups in patients with lung cancer. Plasma levels of VWF and ADAMTS-13, and FVIII activity were measured in 115 patients with lung cancer and 98 healthy subjects...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28912376/recombinant-adamts-13-first-in-human-pharmacokinetics-and-safety-in-congenital-thrombotic-thrombocytopenic-purpura
#16
MULTICENTER STUDY
Marie Scully, Paul Knöbl, Karim Kentouche, Lawrence Rice, Jerzy Windyga, Reinhard Schneppenheim, Johanna A Kremer Hovinga, Michiko Kajiwara, Yoshihiro Fujimura, Caterina Maggiore, Jennifer Doralt, Christopher Hibbard, Leah Martell, Bruce Ewenstein
Safety, tolerability, and pharmacokinetics of recombinant ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; BAX 930; SHP655) were investigated in 15 patients diagnosed with severe congenital ADAMTS-13 deficiency (plasma ADAMTS-13 activity <6%) in a prospective phase 1, first-in-human, multicenter dose escalation study. BAX 930 was well tolerated, no serious adverse events occurred, and no anti-ADAMTS-13 antibodies were observed. After single-dose administration of BAX 930 at 5, 20, or 40 U/kg body weight to adolescents and adults, there was approximate dose proportionality with respect to maximum plasma concentration (Cmax [U/mL]) and area under the concentration-time curve (AUC [h∙U/mL])...
November 9, 2017: Blood
https://www.readbyqxmd.com/read/28843665/balanced-haemostasis-with-both-hypo-and-hyper-coagulable-features-in-critically-ill-patients-with-acute-on-chronic-liver-failure
#17
Caleb Fisher, Vishal C Patel, Sidsel Hyldgaard Stoy, Arjuna Singanayagam, Jelle Adelmeijer, Julia Wendon, Debbie L Shawcross, Ton Lisman, William Bernal
BACKGROUND: Cirrhotic patients have complex haemostatic abnormalities. Current evidence suggests stable cirrhotic (SC) patients have a "re-balanced" haemostatic state. However, limited data exists in acute decompensated (AD) or acute on chronic liver failure (ACLF) patients. METHODS: We utilised thrombin generation analysis, fibrinolysis assessment, and evaluation of haemostatic parameters to assess haemostasis in liver disease of progressive severity...
February 2018: Journal of Critical Care
https://www.readbyqxmd.com/read/28839178/hypercoagulability-progresses-to-hypocoagulability-during-evolution-of-acetaminophen-induced-acute-liver-injury-in-pigs
#18
Karla Chui Luan Lee, Luisa Baker, Susan Mallett, Anne Riddell, Pratima Chowdary, Hatim Alibhai, Yu-Mei Chang, Simon Priestnall, Giacomo Stanzani, Nathan Davies, Rajeshwar Mookerjee, Rajiv Jalan, Banwari Agarwal
Increases in prothrombin time (PT) and international normalised ratio (INR) characterise acute liver injury (ALI) and failure (ALF), yet a wide heterogeneity in clotting abnormalities exists. This study defines evolution of coagulopathy in 10 pigs with acetaminophen (APAP)-induced ALI compared to 3 Controls. APAP administration began at 0 h and continued to 'ALF', defined as INR >3. In APAP pigs, INR was 1.05 ± 0.02 at 0 h, 2.15 ± 0.43 at 16 h and > 3 at 18 ± 1 h. At 12 h thromboelastography (TEG) demonstrated increased clot formation rate, associated with portal vein platelet aggregates and reductions in protein C, protein S, antithrombin and A Disintegrin and Metalloprotease with Thrombospondin type 1 repeats-13 (ADAMTS-13) to 60%, 24%, 47% and 32% normal respectively...
August 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28775254/adamts-13-regulates-neutrophil-recruitment-in-a-mouse-model-of-invasive-pulmonary-aspergillosis
#19
Astrid Alflen, Steve Prüfer, Katharina Ebner, Sebastian Reuter, Pamela Aranda Lopez, Inge Scharrer, Fumiaki Banno, Michael Stassen, Hansjörg Schild, Kerstin Jurk, Markus Bosmann, Hendrik Beckert, Markus P Radsak
Von Willebrand factor (VWF) is secreted as an acute phase protein during inflammation. ADAMTS-13 regulates the size and prothrombotic activity of VWF by it's specific proteolytic activity. To determine the relevance of this regulatory pathway for the innate inflammatory response by polymorphonuclear neutrophils (PMN), we employed a mouse model of invasive pulmonary aspergillosis (IPA) where PMN functionality is crucial for fungal clearance and survival. IPA was induced by intratracheal application of Aspergillus fumigatus (A...
August 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28743060/glucose-fluctuation-and-the-resultant-endothelial-injury-are-correlated-with-pancreatic-%C3%AE-cell-dysfunction-in-patients-with-coronary-artery-disease
#20
Makoto Murata, Hitoshi Adachi, Shigeru Oshima, Masahiko Kurabayashi
AIMS: We evaluated whether glucose fluctuation (GF) causes vascular endothelial injury and affects glucometabolic factors during lengthy oral glucose tolerance test (OGTT). METHODS: We enrolled consecutive 116 patients with coronary artery disease (CAD) who were performed coronary angiography and 4-h OGTT. Blood samples were collected before and 4h after glucose load to measure endothelial injury factor [von Willebrand factor (vWF) and vWF/a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS-13) ratio]...
July 8, 2017: Diabetes Research and Clinical Practice
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