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ADAMTS-13

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https://www.readbyqxmd.com/read/28650450/endothelial-damage-is-aggravated-in-acute-gvhd-and-could-predict-its-development
#1
E Mir, M Palomo, M Rovira, A Pereira, G Escolar, O Penack, E Holler, E Carreras, M Diaz-Ricart
The aim of the present study was to explore whether there is enhanced endothelial dysfunction in patients developing acute GvHD (aGvHD) after allogeneic hematopoietic cell transplantation (allo-HCT) and to identify biomarkers with predictive and/or diagnostic value. In in vitro experiments, endothelial cells (ECs) were exposed to serum from patients with (aGvHD, n=31) and without (NoGvHD, n=13) aGvHD, to evaluate changes in surface adhesion receptors, the reactivity of the extracellular matrix by measuring the presence of Von Willebrand factor (VWF) and platelet adhesion, and the activation of intracellular signaling proteins...
June 26, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#2
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28546076/sirna-knockdown-of-adamts-13-modulates-endothelial-cell-angiogenesis
#3
Huiyuan Tang, Manfai Lee, Eun Ho Kim, Daniel Bishop, George M Rodgers
ADAMTS-13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, is a zinc-containing metalloprotease that cleaves von Willebrand factor (vWf). Previous publications by our laboratory have shown that ADAMTS-13 may also be involved in angiogenesis. For this study, we report the successful transient knockdown of endogenous ADAMTS-13 in human umbilical vein endothelial cells (HUVEC) via siRNA and the effects of reduced endogenous ADAMTS-13 on HUVEC angiogenesis functions. 15nM of ADAMTS-13 siRNA reduced HUVEC ADAMTS-13 protein levels by 90% after 24h incubation, whereas control siRNA did not affect endogenous ADAMTS-13 levels...
May 22, 2017: Microvascular Research
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#4
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28457019/dynamics-of-von-willebrand-factor-reactivity-in-sickle-cell-disease-during-vaso-occlusive-crisis-and-steady-state
#5
J W R Sins, M Schimmel, B M Luken, E Nur, S S Zeerleder, C F J van Tuijn, D P M Brandjes, W F Kopatz, R T Urbanus, J C M Meijers, B J Biemond, K Fijnvandraat
Essentials The role of von Willebrand Factor (VWF) in the pathophysiology of sickle cell disease is unclear. We assessed markers of VWF during admission for vaso-occlusive crisis (VOC) and steady state. VWF reactivity was higher during VOC and was associated with inflammation and neutrophil activation. Hyper-adhesive VWF may promote VOC in sickle cell disease. SUMMARY: Background Endothelial activation plays a central role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), facilitating adhesive interactions with circulating blood cells...
April 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28413093/-thrombotic-thrombocytopenia-purpura-in-martinique-retrospective-study-between-2008%C3%A2-and-2015
#6
M Patient, P Fuseau, C Deligny
INTRODUCTION: Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. METHODS: We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28380082/heparin-induced-thrombocytopenia-as-a-cause-of-prolonged-low-platelet-count-in-a-patient-with-thrombotic-thrombocytopenic-purpura-treated-with-plasmapheresis
#7
Agata Winiarska, Norbert Kwella, Tomasz Stompór
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder belonging to thrombotic microangiopathies (TMA) and is caused by functional deficiency of the ADAMTS-13 metalloproteinase. Plasma exchange (PE) remains the treatment of choice in this disease. Here, were describe the case of a patient who apparently recovered from TTP following multiple sessions of PE, but remained thrombocytopenic. Careful analysis revealed the development of heparin-induced thrombocytopenia (HIT) that precluded platelet count (PLT) normalization...
2017: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/28374895/increased-von-willebrand-factor-over-decreased-adamts-13-activity-is-associated-with-poor-prognosis-in-patients-with-advanced-non-small-cell-lung-cancer
#8
Renyong Guo, Jiezuan Yang, Xia Liu, Jianping Wu, Yu Chen
BACKGROUND: Hypercoagulability induced by the imbalance between von Willebrand factor (VWF) secretion and its cleaving protease (ADAMTS-13) has been correlated with cancer metastasis. The aim of this study was to explore the prognostic significance of the VWF/ADAMTS-13 ratio in advanced non-small-cell lung cancer (NSCLC). METHODS: Pre-treatment sera/plasma levels of VWF, ADAMTS-13, VWF/ADAMTS-13 ratio, factor (F) VIII, and other clinical/laboratory parameters were measured in 119 patients with advanced NSCLC and 102 healthy controls...
April 4, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28325251/interferon-induced-thrombotic-microangiopathy-tma-analysis-and-concise-review
#9
REVIEW
Ajay Kundra, Jen Chin Wang
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome...
April 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28265287/pernicious-anemia-associated-cobalamin-deficiency-and-thrombotic-microangiopathy-case-report-and-review-of-the-literature
#10
Farhanah Yousaf, Bruce Spinowitz, Chaim Charytan, Marilyn Galler
A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28207543/decreased-adamts-13-level-is-related-to-inflammation-factors-and-risk-stratification-of-acute-lymphoblastic-leukemia-patients
#11
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
#12
LETTER
D C Moreira, J Di Paola
No abstract text is available yet for this article.
April 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28176471/subclinical-atypical-haemolytic-uremic-syndrome-relapse-following-discontinuation-of-eculizumab
#13
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
#14
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...
April 2017: Transfusion
https://www.readbyqxmd.com/read/28150184/thrombotic-thrombocytopenic-purpura-in-a-new-onset-lupus-patient
#15
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.
April 2017: Immunologic Research
https://www.readbyqxmd.com/read/28122158/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-comment
#16
LETTER
J Thachil
No abstract text is available yet for this article.
April 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
#17
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
#18
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
#19
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
#20
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...
December 2016: Frontiers of Medicine
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