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ADAMTS-13

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https://www.readbyqxmd.com/read/28207543/decreased-adamts-13-level-is-related-to-inflammation-factors-and-risk-stratification-of-acute-lymphoblastic-leukemia-patients
#1
Chen Liu, Lei Zhao, Jingzhong Zhao, Qinzhu Xu, Ying Song, Hui Wang
As a kind of metalloprotease of the ADAMTS family, ADAMTS-13 is crucial for maintaining the normal size of von Willebrand factor. Reduced ADAMTS-13 had been reported in patients with both localized and disseminated malignancies. However, the expression and potential role of ADAMTS-13 in hematological malignancies remain unclear. In this research, we measured and compared ADAMTS-13 levels in plasma of 35 acute lymphoblastic leukemia (ALL) patients and 30 healthy controls and found that ALL patients possessed lower level of ADAMTS-13 than controls...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28182327/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-reply
#2
Daniel C Moreira, Jorge Di Paola
We sincerely appreciate Dr Thachil's letter, which provides further insight into the case we recently published, in which ADAMTS-13 was likely lost in third-space fluid in a patient with idiopathic systemic capillary leak syndrome (SCLS) and that this loss was proposed as a novel mechanism for microangiopathic hemolytic anemia (MAHA) [1]. As mentioned in the original publication, identifying high levels of ADAMTS-13 in the transudate fluid would have proven the proposed mechanism, but unfortunately a sample of the transudate fluid was not available...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28176471/subclinical-atypical-haemolytic-uremic-syndrome-relapse-following-discontinuation-of-eculizumab
#3
REVIEW
Shi Zhou Choo, Fiona Brown
A 25-year-old man presented with microangiopathic haemolytic anaemia and acute kidney injury. With a normal ADAMTS-13 level, negative faecal shiga-toxin test and strong family history of atypical haemolytic uremic syndrome, he was commenced on eculizumab to good clinical response. Subsequent genetic testing revealed a heterozygous complement factor H mutation. Eculizumab was discontinued after 44 months of treatment, and he relapsed within 6 months, with the first sign being downtrending haptoglobin levels, with no other markers of haemolysis or thrombocytopaenia, 5 weeks prior to development of acute kidney injury...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28150310/plasma-treated-with-amotosalen-and-ultraviolet-a-light-retains-activity-for-hemostasis-after-5-days-post-thaw-storage-at-1-to-6-o-c
#4
Anna Erickson, Katie Waldhaus, Tovo David, Norman Huang, Salvador Rico, Laurence Corash, Nina Mufti, Richard J Benjamin
BACKGROUND: Plasma thawed and stored at 1 to 6(°) C for up to 5 days (thawed plasma [TP]) provides rapid availability in emergencies and reduces plasma waste, but it carries risks of coagulation factor loss or activation, bacterial outgrowth, and viral contamination. We characterized changes in amotosalen/ultraviolet A (UVA) light pathogen-reduced, fresh-frozen plasma (FFP) and plasma frozen within 24 hours (PF24) with post-thaw storage. STUDY DESIGN AND METHODS: Amotosalen/UVA light-treated FFP and PF24 were thawed after approximately 3 to more than 12 months of frozen storage and held at 1 to 6(°) C for 5 days...
February 1, 2017: Transfusion
https://www.readbyqxmd.com/read/28150184/thrombotic-thrombocytopenic-purpura-in-a-new-onset-lupus-patient
#5
Nery Sablón González, Noel Lorenzo, Yanet Parodis, Maria Belen Alonso Ortiz, Melek Kechida, Jose Carlos Rodriguez Perez
We are presenting a case of TTP with undetectable levels of ADAMTS 13 in a 39-year-old woman. Diagnosis of systemic lupus was evoked in the setting of thrombotic microangiopathy. The patient presented normal renal function but important neurological impairment. Treatment with daily plasmapheresis as well as Rituximab, cyclophosphamide as steroids was required to achieve clinical improvement.
February 2, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28122158/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-comment
#6
Jecko Thachil
Moreira et al describe a fascinating case of a five-year-old who developed microangiopathic hemolytic anemia (MAHA) a week after presentation with idiopathic systemic capillary leak syndrome (SCLS) and propose ADAMTS-13 loss in the third-space fluid as the cause of MAHA [1]. It would however be useful if the authors could provide the values of vascular endothelial growth factor (VEGF) and von willebrand factor (VWF) levels in this patient during the illness episode before this conclusion is made. Endothelial dysfunction is well-known to cause very high levels of both VEGF and VWF, to the extent in many experimental studies; these markers are used as a marker for endothelial activation and altered function [2, 3]...
January 25, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28088271/adamts-3-13-16-and-19-levels-in-patients-with-habitual-abortion
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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