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ADAMTS-13

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https://www.readbyqxmd.com/read/28088271/adamts-3-13-16-and-19-levels-in-patients-with-habitual-abortion
#1
Meryem Kuru Pekcan, Esma Sarıkaya, Aytekin Tokmak, Murat Alışık, Afra Alkan, Gülnur Özakşit, Özcan Erel
A disintegrin-like and metalloproteinase domain with thrombospondin-type 1 motifs (ADAMTS) protein superfamily includes 19 secreted metalloproteases. Proteolytic substrates of ADAMTS enzymes have been linked to reproductive function. The aim of this study was to investigate serum ADAMTS-3, -13, -16, and -19 levels in women with habitual abortions compared with those in healthy controls. A total of 86 women were enrolled in this prospective case-control study. ADAMTS-3, -13, -16, and -19 values were recorded and analyzed in association with demographic and clinical parameters...
January 2017: Kaohsiung Journal of Medical Sciences
https://www.readbyqxmd.com/read/28033504/reduced-adamts-13-level-negatively-correlates-with-inflammation-factors-in-plasma-of-acute-myeloid-leukemia-patients
#2
Chen Liu, Lei Zhao, Jingzhong Zhao, Qinzhu Xu, Ying Song, Hui Wang
ADAMTS-13 is crucial for maintaining the normal size of vWF. Besides thrombotic thrombocytopenic purpura (TTP), decreased ADAMTS-13 had also been reported in patients with malignancy. However, the knowledge of expression and potential role of ADAMTS-13 in hematological malignancies is still limited. We measured and compared ADAMTS-13 levels in the plasma of 82 acute myeloid leukemia (AML) patients and 34 healthy controls and found that AML patients possessed lower ADAMTS-13 than controls. AML patients with infections possessed lower level of ADAMTS-13 than patients without infections and ADAMTS-13 levels were negatively correlated with C-reactive protein(CRP), IL-6, TNFα and IL-1β...
December 20, 2016: Leukemia Research
https://www.readbyqxmd.com/read/27940692/severe-hemolysis-and-pulmonary-hypertension-in-a-neonate-with-upshaw-schulman-syndrome
#3
Nobuyuki Tsujii, Isao Shiraishi, Koichi Kokame, Midori Shima, Yoshihiro Fujimura, Yukihiro Takahashi, Masanori Matsumoto
Pulmonary involvement is extremely rare in thrombotic thrombocytopenic purpura. In this report, we present a girl patient with congenital thrombotic thrombocytopenic purpura, known as Upshaw-Schulman syndrome (USS), complicated with severe hemolysis and pulmonary hypertension (PH). The assay results of a disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS13) activity measured by FRETS-VWF73 and ADAMTS13-act-ELISA were different. Hyperbilirubinemia (total bilirubin, 25.3 mg/dL) interfered strongly with the FRETS-VWF73 assay...
December 2016: Pediatrics
https://www.readbyqxmd.com/read/27878664/diagnosis-and-management-of-acquired-thrombotic-thrombocytopenic-purpura-in-southeast-china-a-single-center-experience-of-60-cases
#4
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/27807630/-role-of-plasmapheresis-and-immunoadsorption-in-salvage-therapy-of-rheumatological-diseases
#5
M Boser, J T Kielstein
Many rheumatological diseases are either caused by specific known proteins, such as antibodies or mediated by a plethora of cytokines. Both the unspecific immunosuppressive therapy and the specific action of biologics usually require time to be effective; therefore, extracorporeal forms of treatment are increasingly being employed in severe forms of rheumatological diseases as well as in patients who cannot tolerate pharmacological treatment or where the risk of pharmacological treatment may outweigh the potential benefits...
December 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#6
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27717422/left-ventricular-assist-device-design-reduces-von-willebrand-factor-degradation-a-comparative-study-between-the-heartmate-ii-and-the-evaheart-left-ventricular-assist-system
#7
Carlo R Bartoli, Jooeun Kang, David Zhang, Jessica Howard, Michael Acker, Pavan Atluri, Tadashi Motomura
BACKGROUND: Supraphysiologic shear stress from continuous-flow left ventricular assist devices (LVADs) accelerates von Willebrand factor (vWF) degradation and predisposes patients to nonsurgical bleeding. It is unknown whether unique design characteristics of LVADs differentially affect vWF degradation. We tested the hypothesis that the centrifugal-flow EVAHEART (Evaheart, Houston, TX) left ventricular assist system (LVAS), which was designed to minimize shear stress (low operational revolutions per minute [rpm], larger flow gaps, low shear stress, flat H-Q curve), reduced vWF degradation versus the axial-flow HeartMate II (Thoratec, Pleasanton, CA) LVAD...
October 4, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27622772/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome
#8
D C Moreira, C J Ng, R Quinones, X Liang, D W Chung, J Di Paola
: Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. SUMMARY: Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema...
December 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27621680/hematopoietic-cell-transplantation-associated-thrombotic-microangiopathy-a-review-of-pathophysiology-diagnosis-and-treatment
#9
REVIEW
Joseph Rosenthal
Transplant-associated thrombotic microangiopathy (TA-TMA) is a multifactorial disorder caused by systemic vascular endothelial injury that can be triggered by several mechanisms during the transplant process. Thrombotic microangiopathy may affect multiple systems and occurs in ~30% of patients undergoing hematopoietic stem cell transplantation. A subgroup of patients with thrombotic microangiopathy develop TA-TMA, and the other may develop other thrombotic microangiopathic disorders such as thrombotic thrombocytopenic purpura, a condition with similar finding but different pathophysiology involving ADAMTS-13...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27613177/evaluation-of-von-willebrand-factor-and-adamts-13-antigen-and-activity-levels-in-sickle-cell-disease-patients-in-kuwait
#10
Anwar Al-Awadhi, Adekunle Adekile, Rajaa Marouf
Sickle cell disease (SCD) is a severe form of hemolytic anemia characterized by chronic hemolysis and is associated with increased thrombotic risk. Elevated von Willebrand factor (vWF) levels in SCD have been attributed to increased secretion and impaired processing by its cleaving protease ADAMTS-13. In this study we measured vWF and ADAMTS-13 antigen and activity levels in our SCD patients. Hematological and biochemical parameters for 59 SCD patients (20 children and 39 adults) were analyzed and compared to 59 age- and sex-matched controls...
September 9, 2016: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/27559008/low-adamts-13-activity-and-the-risk-of-coronary-heart-disease-a-prospective-cohort-study-the-rotterdam-study
#11
M A H Sonneveld, M Kavousi, M A Ikram, A Hofman, O L Rueda Ochoa, P L Turecek, O H Franco, F W G Leebeek, M P M de Maat
: Essentials An association between ADAMTS-13 and coronary heart disease (CHD) has been suggested. 5688 participants ≥ 55 years from the Rotterdam Study without a history of CHD were included. Over a median follow-up time of 9.7 years, 456 individuals suffered from CHD. Low ADAMTS-13 activity was associated with an increased CHD risk. SUMMARY: Background The metalloprotease ADAMTS-13 cleaves high-molecular-weight von Willebrand factor multimers into smaller, less procoagulant forms...
August 25, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27545639/-efficacy-of-eculizumab-in-a-case-of-pregnancy-associated-ahus
#12
Raffaella Cravero, Gianluigi Ardissino, Umberto Colageo, Piernicola Staffa, Elena Bruschetta, Serena Maroni, Eirini Karvela, Ermanno Spagarino, Velia Ruggeri, Roberto Jura
Pregnancy-associated thrombotic microangiopathy (TMA) is a rare condition, but it is burdened by a significant perinatal and maternal morbidity as well as mortality. We describe the case of a 33-year-old woman, who developed a TMA at the 36th week of gestation characterized by increased LDH, haptoglobin consumption, schistocytes, thrombocytopenia and acute renal failure requiring dialysis. There were not gestational hypertension nor proteinuria until the day of hospitalization. ADAMTS 13 deficiency was ruled out and the patient did not have diarrhea...
July 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27435143/validation-of-a-panel-of-adamts13-assays-for-diagnosis-of-thrombotic-thrombocytopenic-purpura-activity-functional-inhibitor-and-autoantibody-test
#13
M O Nakashima, X Zhang, H J Rogers, L Vengal, B Gibson, T M Daly, K Kottke-Marchant
INTRODUCTION: Quantitation of ADAMTS13 activity, functional inhibitors, and autoantibodies is crucial in diagnosis and management of thrombotic thrombocytopenic purpura. We compared and optimized commercial assay kits and validated a testing panel. METHODS: Citrated plasma specimens from healthy volunteers and residual samples submitted for clinical testing were used in the study. Commercially available ADAMTS13 activity assays including ACTIFLUOR(™) ADAMTS13 (Sekisui Diagnostics, Stamford, CT, USA), LIFECODES ATS-13 (Gen-Probe Inc...
October 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27371116/preclinical-assessment-of-a-new-recombinant-adamts-13-drug-product-bax930-for-the-treatment-of-thrombotic-thrombocytopenic-purpura
#14
A Kopić, K Benamara, C Piskernik, B Plaimauer, F Horling, G Höbarth, T Ruthsatz, B Dietrich, E-M Muchitsch, F Scheiflinger, M Turecek, W Höllriegl
UNLABELLED: Essentials ADAMTS-13-deficiency is a cause of thrombotic thrombocytopenic purpura (TTP). Preclinical safety of recombinant human ADAMTS-13 (BAX930) was shown in animal models. Preclinical efficacy of BAX930 was shown in a mouse model of TTP. BAX930 showed advantageous efficacy over fresh frozen plasma, the current standard of care. Click to hear Dr Cataland and Prof. Lämmle present a seminar on Thrombotic Thrombocytopenic Purpura (TTP): new Insights in Pathogenesis and Treatment Modalities...
July 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27246502/two-approaches-to-the-clinical-dilemma-of-treating-ttp-with-therapeutic-plasma-exchange-in-patients-with-a-history-of-anaphylactic-reactions-to-plasma
#15
Davinder Sidhu, Edward L Snyder, Christopher A Tormey
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare but serious disease caused by autoantibody-mediated deficiency in von Willebrand factor (VWF) cleaving protease, ADAMTS-13. The primary acute treatment is therapeutic plasma exchange (TPE). However, some patients can develop allergic/anaphylactic reactions to the replacement (i.e., donor) plasma over time. Two potential treatment strategies for patients with TTP who demonstrate severe allergic reactions to plasma used for exchange were examined...
June 1, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27220954/presence-of-portal-vein-thrombosis-in-liver-cirrhosis-is-strongly-associated-with-low-levels-of-adamts-13-a-pilot-study
#16
Stefano Lancellotti, Maria Basso, Vito Veca, Monica Sacco, Laura Riccardi, Maurizio Pompili, Raimondo De Cristofaro
Portal vein thrombosis (PVT) dramatically changes the prognosis of cirrhotic patients, especially those waiting for liver transplantation. However, the possible contribution to PVT of von Willebrand factor (VWF) and ADAMTS-13 is poorly documented. The aim of our study was to assess the presence of alterations of VWF and ADAMTS-13 serum levels in cirrhotic patients with PVT. Twenty-four patients with PVT (group PVT) and 60 without PVT (group without PVT) were enrolled. A comprehensive analysis of biochemical and hemostatic parameters was performed...
October 2016: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/27195703/coombs-positive-thrombotic-thrombocytopenic-purpura-in-a-male-pediatric-patient-an-urgent-diagnostic-challenge
#17
Anna Zenno, Matthew Richardson
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy often caused by deficiency of von Willebrand (vW) factor cleaving protease, ADAMTS-13, leading to large vW multimers and intravascular platelet aggregation. Hemolysis in TTP is mechanical and nonimmune mediated, thus Coombs testing is usually negative. We report a case of an adolescent with thrombocytopenia and Coombs positive anemia, diagnosed with Evans syndrome, but ultimately found to have TTP. TTP should be considered in children with thrombocytopenia and Coombs positive anemia who are refractory to steroids or develop signs of microangiopathy...
October 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27181025/tissue-plasminogen-activator-neurotoxicity-is-neutralized-by-recombinant-adamts-13
#18
Mengchen Fan, Haochen Xu, Lixiang Wang, Haiyu Luo, Ximin Zhu, Ping Cai, Lixiang Wei, Lu Lu, Yongliang Cao, Rong Ye, Wenying Fan, Bing-Qiao Zhao
Tissue plasminogen activator (tPA) is an effective treatment for ischemic stroke, but its neurotoxicity is a significant problem. Here we tested the hypothesis that recombinant ADAMTS 13 (rADAMTS 13) would reduce tPA neurotoxicity in a mouse model of stroke. We show that treatment with rADAMTS 13 in combination with tPA significantly reduced infarct volume compared with mice treated with tPA alone 48 hours after stroke. The combination treatment significantly improved neurological deficits compared with mice treated with tPA or vehicle alone...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27166361/clinical-and-prognostic-significance-of-serum-levels-of-von-willebrand-factor-and-adamts-13-antigens-in-al-amyloidosis
#19
Efstathios Kastritis, Ioannis Papassotiriou, Evangelos Terpos, Maria Roussou, Maria Gavriatopoulou, Anna Komitopoulou, Chrysanthi Skevaki, Evangelos Eleutherakis-Papaiakovou, Constantinos Pamboucas, Erasmia Psimenou, Efstathios Manios, Stavroula Giannouli, Marianna Politou, Harikleia Gakiopoulou, Elektra Papadopoulou, Kimon Stamatelopoulos, Anna Tasidou, Meletios A Dimopoulos
Cardiac dysfunction determines prognosis in amyloid light-chain (AL) amyloidosis. The heart is the central organ of the vascular system in which endothelium function is critical for the circulatory homeostasis, but there are limited data on endothelial function in AL amyloidosis. von Willebrand factor (VWF) has been considered as a marker of endothelial activation and dysfunction, whereas a disintegrin and metalloproteinase with thrombospondin type-1 repeats 13 (ADAMTS-13) cleaves VWF multimers, but both have been associated with prognosis in cardiovascular disease...
July 21, 2016: Blood
https://www.readbyqxmd.com/read/27151031/-influence-of-hsct-preconditioning-on-adamts-13-activity-and-vwf-level-and-its-clinical-significance
#20
Jie Wang, Yue Han, Lu-Ping Hu, De-Pei Wu, Chang-Geng Ruan
OBJECTIVE: To explore the influence of precondintioning on ADAMTS-13 activity and vWF level, and its clinical significance by measuring the alterations of ADAMTS-13 activity and vWF antigen levels before and after preconditioning of hematopoietic stem cell transplantation (HSCT) in the patients. METHOD: A total of 113 patients received HSCT in the First Hospital affiliated to Soochow University were investigated, 20 healthy volunteers were used as the control. The ADAMTS-13 activity and vWF antigen level were measured by FRETS-vWF73 and ELISA respectively...
April 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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