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https://www.readbyqxmd.com/read/27904906/what-adamts13-does-in-the-liver%C3%A2
#1
Johann Wojta
No abstract text is available yet for this article.
December 1, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27898514/a-rare-combination-of-thrombotic-thrombocytopenic-purpura-and-antiphospholipid-syndrome
#2
Maya Viner, Irina Murakhovskaya
Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27891624/quarantine-versus-pathogen-reduced-plasma-coagulation-factor-content-and-rotational-thromboelastometry-coagulation
#3
Oliver M Theusinger, David Goslings, Jan-Dirk Studt, Brigitte Brand-Staufer, Burkhardt Seifert, Donat R Spahn, Beat M Frey
BACKGROUND: Different types of fresh-frozen plasma (FFP) exist, and the concentrations of plasma proteins vary between individuals and blood groups. Furthermore, processing may also influence the content. Quarantine-stored plasma (qFFP) and plasma that was pathogen-reduced using blood-safety (Intercept) technology (piFFP) were analyzed regarding procoagulant and anticoagulant hemostasis proteins, including endogenous thrombin (thrombin-generation) potential (ETP). MATERIALS AND METHODS: Thirty-five samples of each type of FFP were analyzed using only male Blood Group O donors...
November 27, 2016: Transfusion
https://www.readbyqxmd.com/read/27887777/adamts13-specific-circulating-immune-complexes-as-potential-predictors-of-relapse-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#4
Ilaria Mancini, Barbara Ferrari, Carla Valsecchi, Silvia Pontiggia, Marco Fornili, Elia Biganzoli, Flora Peyvandi
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy due to the development of autoantibodies against the VWF-cleaving protease ADAMTS13. ADAMTS13-specific circulating immune complexes (CICs) have been described in patients with acquired TTP, but their clinical relevance remained to be established. The aim of this study was to assess the association between ADAMTS13-specific CICs and ADAMTS13-related measurements, clinical and laboratory markers of disease severity, and occurrence of TTP relapse, in autoimmune TTP patients...
November 22, 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27884834/glycans-of-plasma-adamts13
#5
Karen Vanhoorelbeke
No abstract text is available yet for this article.
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27879275/von-willebrand-factor-and-adamts13-too-much-or-too-little-of-a-good-thing
#6
EDITORIAL
Brian T Emmer, David Ginsburg, Karl C Desch
No abstract text is available yet for this article.
December 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/27878664/diagnosis-and-management-of-acquired-thrombotic-thrombocytopenic-purpura-in-southeast-china-a-single-center-experience-of-60-cases
#7
Xinping Zhou, Xingnong Ye, Yanling Ren, Chen Mei, Liya Ma, Jiansong Huang, Weilai Xu, Juying Wei, Li Ye, Wenyuan Mai, Wenbin Qian, Haitao Meng, Jie Jin, Hongyan Tong
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1...
November 23, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27866840/the-role-of-human-leukocyte-antigen-drb1-dqb1-haplotypes-in-the-susceptibility-to-acquired-idiopathic-thrombotic-thrombocytopenic-purpura
#8
György Sinkovits, Ágnes Szilágyi, Péter Farkas, Dóra Inotai, Anikó Szilvási, Attila Tordai, Katalin Rázsó, Marienn Réti, Zoltán Prohászka
The acquired form of idiopathic thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease, in which the underlying ADAMTS13-deficiency is caused by inhibitory autoantibodies against the protease. Human leukocyte antigens (HLA), responsible for antigen presentation, play an important role in the development of antibodies. The loci coding HLA DR and DQ molecules are inherited in linkage as haplotypes. The c.1858C>T polymorphism of the PTPN22 gene, which codes a protein tyrosine phosphatase important in lymphocyte activation, predisposes to a number of autoimmune diseases...
November 17, 2016: Human Immunology
https://www.readbyqxmd.com/read/27859681/familial-acquired-thrombotic-thrombocytopenic-purpura-in-siblings-no-immunogenetic-link-with-associated-human-leukocyte-antigens
#9
Philipp Gödel, Julia Fischer, Christoph Scheid, Birgit S Gathof, Jürgen Wolf, Jan Rybniker
Acquired immunoglobulin G (IgG) mediated thrombotic thrombocytopenic purpura (TTP) has not yet been described in non-twin siblings. We report two cases of acquired TTP in Caucasian sisters with inactive ADAMTS13 metalloprotease due to ADAMTS13 autoantibodies suggesting a role of genetic determinants in this life-threatening disease. However, human leukocyte antigen (HLA) class II types presumably associated with acquired thrombotic thrombocytopenic purpura were not identified in the patients, indicating that HLA class II typing may not be useful in acquired TTP risk assessment of family members...
November 18, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27822882/plasma-von-willebrand-factor-levels-predict-in-hospital-survival-in-patients-with-acute-on-chronic-liver-failure
#10
K S Prasanna, Ashish Goel, G Jayakumar Amirtharaj, Anup Ramachandran, K A Balasubramanian, Ian Mackie, Uday Zachariah, K G Sajith, Elwyn Elias, C E Eapen
BACKGROUND AND AIMS: Circulating levels of von Willebrand factor (vWF) predict mortality in patients with cirrhosis. We hypothesized that systemic inflammation in acute-on-chronic liver failure (ACLF) will stimulate endothelium, increase vWF levels, and promote platelet microthrombi causing organ failure. METHODS: In this prospective study, we correlated plasma vWF levels with organ failure, liver disease severity, sepsis, and systemic inflammatory response syndrome (SIRS) and also analyzed if vWF levels predicted in-hospital composite poor outcome (i...
November 8, 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/27807280/blood-biomarkers-associated-with-neurological-deterioration-in-patients-with-acute-penetrating-artery-territory-infarction-a-multicenter-prospective-observational-study
#11
Tomohiro Kawano, Kotaro Miyashita, Mariko Takeuchi, Yoshinari Nagakane, Yasumasa Yamamoto, Kenji Kamiyama, Yasuhiro Manabe, Kenichi Todo, Norifumi Metoki, Yasuhisa Akaiwa, Kazunori Toyoda, Kazuyuki Nagatsuka
BACKGROUND AND PURPOSE: Neurological deterioration in acute penetrating artery territory infarction is unpredictable and associated with unfavorable clinical outcomes. The aim of this prospective study was to clarify the cause of neurological worsening and predict clinical outcomes using blood biomarkers. METHODS: Eight Japanese stroke centers participated. Blood samples were obtained within 24 h (the first sampling) and on day 7 in hospital (the second sampling) in patients with penetrating artery territory infarction, arriving within two days of stroke onset...
November 2, 2016: International Journal of Stroke: Official Journal of the International Stroke Society
https://www.readbyqxmd.com/read/27802307/next-generation-sequencing-and-in-vitro-expression-study-of-adamts13-single-nucleotide-variants-in-deep-vein-thrombosis
#12
Maria Teresa Pagliari, Luca A Lotta, Hugoline G de Haan, Carla Valsecchi, Gloria Casoli, Silvia Pontiggia, Ida Martinelli, Serena M Passamonti, Frits R Rosendaal, Flora Peyvandi
BACKGROUND: Deep vein thrombosis (DVT) genetic predisposition is partially known. OBJECTIVES: This study aimed at assessing the functional impact of nine ADAMTS13 single nucleotide variants (SNVs) previously reported to be associated as a group with DVT in a burden test and the individual association of selected variants with DVT risk in two replication studies. METHODS: Wild-type and mutant recombinant ADAMTS13 were transiently expressed in HEK293 cells...
2016: PloS One
https://www.readbyqxmd.com/read/27795521/von-willebrand-factor-and-von-willebrand-disease
#13
Taei Matsui, Jiharu Hamako
von Willebrand factor (VWF) has two major roles in hemostasis, as a form of molecular glue which functions in platelet plug formation and as a protective transporter for coagulation factor VIII (FVIII). VWF shows a multimeric chain structure composed of 270 kDa subunits containing binding domains for FVIII, platelet and collagens. Biosynthesis, storage, secretion of VWF and the cleavage process by ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 repeats 13) regulating VWF activity have been elucidated...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27787621/adamts13-activity-as-a-novel-risk-factor-for-incident-type-2-diabetes-mellitus-a-population-based-cohort-study
#14
Paul S de Vries, Thijs T W van Herpt, Symen Ligthart, Albert Hofman, M Arfan Ikram, Mandy van Hoek, Eric J G Sijbrands, Oscar H Franco, Moniek P M de Maat, Frank W G Leebeek, Abbas Dehghan
AIMS/HYPOTHESIS: ADAMTS13 is a protease that breaks down von Willebrand factor (VWF) multimers into smaller, less active particles. VWF has been associated with an increased risk of incident type 2 diabetes mellitus. Here, we determine whether ADAMTS13 activity and VWF antigen are associated with incident diabetes. METHODS: This study included 5176 participants from the Rotterdam Study, a prospective population-based cohort study. Participants were free of diabetes at baseline and followed up for more than 20 years...
October 27, 2016: Diabetologia
https://www.readbyqxmd.com/read/27784345/-changes-of-adamts13-activity-and-tsp1-level-in-patients-with-hematologic-malignancies
#15
Cai-Feng Sun, Xia Zhao, Fang Han, Qi Jia, Liang Wang, Guang Lu, Hui-Fang Ding
OBJECTIVE: To investigate the changes of thrombospondin 1(TSP1) level and von Willebrand factor cleaving protease(ADAMTS13) activity in the patients with hematologic malignancies before and after treatment and to evaluate their clinical significance. METHODS: Eighty-two patients with hematologic malignancies were enrolled in this study, among them 20 patients were with acute leukemia, 48 patients were with lymphoma and 14 patients were with multiple myeloma. The plasma samples of 82 patients with hematologic malignancies and 45 healthy controls were collected...
October 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27777394/systemic-lupus-erythematosus-presenting-as-refractory-thrombotic-thrombocytopenic-purpura-a-diagnostic-and-management-challenge-a-case-report-and-concise-review-of-the-literature
#16
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27775781/clinical-significance-of-dynamic-changes-in-hs-crp-and-adamts13-levels-in-the-blood-serum-of-patients-with-no-reflow-after-pci-operation
#17
W B Liu, Z-P Zou, H-P Jiang, Q Li, F-M Guo, Z Wang, Z-S Bi
OBJECTIVE: To evaluate the clinical significance dynamic changes in hs-CRP and ADAMTS13 levels in patients with no-reflow after PCI operation. PATIENTS AND METHODS: 156 patients with STEMI single-vessel were enrolled in this study. Patients were divided into the reflow and the no-reflow groups. ADAMTS13 and hs-CRP levels were compared between the two groups. RESULTS: ADAMTS13 and hs-CRP levels in the no-reflow group were significantly lower than those in the reflow group after PCI operation...
October 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27771173/complement-activation-inflammation-and-relative-adamts13-deficiency-in-secondary-thrombotic-microangiopathies
#18
Péter Farkas, Dorottya Csuka, Bálint Mikes, György Sinkovits, Marienn Réti, Endre Németh, Kristóf Rácz, Krisztina Madách, Mihály Gergely, Judit Demeter, Zoltán Prohászka
BACKGROUND: The secondary forms of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (secondary TMA) emerge as complications of coexisting diseases. OBJECTIVES: We hypothesized that secondary TMA could be characterized by the presence of relative ADAMTS13 deficiency and complement activation, and this relationship may have a prognostic value for outcome. PATIENTS AND METHODS: Fifty-three patients with thrombotic microangiopathy (TMA) and coexisting disease (such as malignancies, sepsis, heart surgery with extracorporeal circulation, solid organ transplantation, systemic autoimmune disorders), 41 patient controls, and 34 healthy controls were enrolled in our case-control study with 30days follow-up...
October 18, 2016: Immunobiology
https://www.readbyqxmd.com/read/27766045/clinical-evaluation-of-thrombotic-microangiopathy-identification-of-patients-with-suspected-atypical-hemolytic-uremic-syndrome
#19
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27766025/tissue-factor-a-potent-stimulator-of-von-willebrand-factor-synthesis-by-human-umbilical-vein-endothelial-cells
#20
Muriel Meiring, W Allers, E Le Roux
Inflammation and dysfunction of endothelial cells are thought to be triggers for the secretion of Von Willebrand factor. The aim of this study was to examine the effects of the inflammatory cytokines interleukin-6 (IL-6), interleukin-8 (IL-8) and tumour necrosis factor-alpha (TNF-α) and the coagulation factors, tissue factor and thrombin on the release and cleavage potential of ultra-large von Willebrand factor (ULVWF) and its cleavage protease by cultured human umbilical vein endothelial cells (HUVEC). HUVEC were treated with IL-6, IL-8, and TNF-α, tissue factor (TF) and thrombin, and combinations thereof for 24 hours under static conditions...
2016: International Journal of Medical Sciences
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