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ADAMTS13

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https://www.readbyqxmd.com/read/29152610/mutations-in-coagulation-factor-viii-are-associated-with-more-favorable-outcome-in-patients-with-cutaneous-melanoma
#1
Zheng Ping, Abha Soni, Lance A Williams, Huy P Pham, Malay K Basu, X Long Zheng
Coagulation factor VIII (FVIII), von Willebrand factor (VWF), and ADAMTS13 play an important role in regulation of normal hemostasis. However, little is known about their roles in patients with malignancy, particularly with cutaneous melanoma. Whole genome sequencing data are available for 25,719 cases in 126 cancer genomic studies for analysis. All sequencing data and corresponding pathology findings were obtained from The Cancer Genome Atlas. The cBioportal bioinformatics tools were used for the data analysis...
July 2017: TH Open
https://www.readbyqxmd.com/read/29150875/treatment-of-acquired-thrombotic-thrombocytopenic-purpura-in-the-u-s-remains-heterogeneous-current-and-future-points-of-clinical-equipoise
#2
Marshall A Mazepa, Jay S Raval, Mark E Brecher, Yara A Park
BACKGROUND: The purpose of this survey was to describe current practices in the U.S. for treatment of acquired Thrombotic Thrombocytopenic Purpura (TTP), compare these with prior U.S. and current Canadian practices, and identify areas of clinical equipoise. STUDY DESIGN AND METHODS: A research team member administered the survey by telephone. Questions included an estimate of the annual patient volume treated, apheresis and medical therapy practices for acquired TTP...
November 18, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#3
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29123569/diagnostic-dilemma-severe-thrombotic-microangiopathy-in-pregnancy
#4
Sarah Birkhoelzer, Alexandra Belcher, Helen Peet
A diagnostic dilemma occurred when thrombotic microangiopathy developed during pregnancy. The diagnostic criteria of thrombotic microangiopathy include thrombocytopenia (platelets <100) and microangiopathic haemolytic anaemia (including thrombotic thrombocytopenic purpura and haemolytic-uraemic syndrome). An urgent interdisciplinary approach is required to treat thrombotic microangiopathy in pregnancy to differentiate between thrombotic microangiopathy and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets)...
November 2017: J Intensive Care Soc
https://www.readbyqxmd.com/read/29121438/platelet-rescue-by-macrophage-depletion-in-obese-adamts13-deficient-mice-at-risk-for-thrombotic-thrombocytopenic-purpura
#5
Lotte Geys, Elien Roose, Ilse Scroyen, Hanspeter Rottensteiner, Claudia Tersteeg, Marc F Hoylaerts, Karen Vanhoorelbeke, H Roger Lijnen
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the absence of ADAMTS13 activity. Thrombocytopenia is presumably related to the formation of microthrombi rich in von Willebrand Factor (VWF) and platelets. Obesity may be a risk factor for TTP; it is associated with abundance of macrophages that may phagocytose platelets. OBJECTIVES: To evaluate the role of obesity and ADAMTS13 deficiency in TTP, and to establish whether macrophages contribute to thrombocytopenia...
November 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29108103/adamts13-and-von-willebrand-factor-a-dynamic-duo
#6
REVIEW
Kieron South, David A Lane
VWF is a key player in haemostasis, acting as a carrier for FVIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase, ADAMTS13. Recently, it has been shown that ADAMTS13 undergoes a conformational change upon interaction with VWF and that this enhances its activity towards its substrate. This review summarises recent work on these conformational transitions, describing how they are controlled...
November 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29106071/multiple-centre-evaluation-study-of-adamts13-activity-and-inhibitor-assays
#7
K Langley, R Fretwell, S Kitchen, S MacDonald, T Dutt, P Baker, D Singh, V McDonald, C Hughes, P Murphy, M Scully
INTRODUCTION: Accurate evaluation of ADAMTS13 activity is required for the diagnosis and clinical management of thrombotic microangiopathies, and commercial kits are available for routine laboratory use. METHODS: Our study compares the results from Technoclone (Technoclone GmbH, Austria) activity and Inhibitor kits with specialist laboratory reference methods (FRETS and ELISA IgG) and the impact of transporting frozen samples and comparison of results. RESULTS: This multicentre study identified differences in Technoclone activity results compared to specialist testing, which could potentially impact diagnosis...
November 6, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29080561/differential-diagnosis-of-thrombotic-microangiopathy-in-nephrology
#8
T Sakari Jokiranta, Ondrej Viklicky, Saleh Al Shorafa, Rosanna Coppo, Christoph Gasteyger, Manuel Macia, Tatiana Pankratenko, Mohan Shenoy, Oğuz Soylemezoglu, Michel Tsimaratos, Jack Wetzels, Hermann Haller
BACKGROUND: The differential diagnosis of thrombotic microangiopathy (TMA) is complex however the rapid diagnosis of the underlying condition is vital to inform urgent treatment decisions. A survey was devised with the objective of understanding current practices across Europe and the Middle East, and of challenges when diagnosing the cause of TMA. METHODS: Over 450 clinicians, from 16 countries were invited to complete an online survey. RESULTS: Of 254 respondents, the majority were nephrologists, had >10 years' experience in their specialty, and had diagnosed a patient with TMA...
October 28, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29077161/expression-and-significance-of-vwf-gmp-140-and-adamts13-in-patients-with-aneurysmal-subarachnoid-hemorrhage
#9
W-H Li, C-J Hui, H Ju
OBJECTIVE: The aim of this study was to investigate the role of the von Willebrand factor (vWF) in patients with aneurysmal subarachnoid hemorrhage (aSAH), platelet membrane glycoprotein-140 (GMP-140). The aim is also to discover the expression and clinical significance of von Willebrand factor (vWF) cleaving protease (ADAMTS13). PATIENTS AND METHODS: 83 patients with aSAH were selected from January 2014 to December 2016. The patients were divided into cerebral vasospasm group (CVS group) (n = 37) and no convulsion group (non-CVS group) (n = 46); delayed cerebral ischemia group (DCI group) (n = 31) and non-delayed cerebral ischemia group (non-DCI group) (n = 52)...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29067443/role-of-adamts13-in-diet-induced-liver-steatosis
#10
Lotte Geys, Elien Roose, Karen Vanhoorelbeke, Pierre Bedossa, Ilse Scroyen, H Roger Lijnen
Previous studies, predominantly based on increased or decreased plasma levels, have reported conflicting data on a potential functional role of ADAMTS13 in the pathogenesis of liver diseases, including non‑alcoholic steatohepatitis (NASH). The aim of the current study was to evaluate whether ADAMTS13 deficiency affects development of NASH. Therefore, male wild‑type (WT) and Adamts13 deficient (Adamts13‑/‑) mice were kept on a steatosis‑inducing diet devoid of methionine and choline (MCD) or a control diet (MCC) for 4 weeks...
August 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29040872/highly-elevated-plasma-level-of-von-willebrand-factor-accelerates-the-formation-of-platelet-thrombus-under-high-shear-stress-in-plasma-with-deficient-adamts13-activity
#11
Hideo Yagi, Naoko Yamaguchi, Yasuaki Shida, Mitsuhiko Sugimoto, Kazuo Tubaki, Yoshihiro Fujimura, Masanori Matsumoto
Upshaw-Schulman syndrome (USS) is a thrombo-hemorrhagic disease caused by congenital deficiency of ADAMTS13 due to ADAMTS13 gene mutations. USS is characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond dramatically to infusions of fresh frozen plasma. There are two phenotypic expressions of USS: one is the early-onset type and the other, the late-onset type, is asymptomatic during childhood with the first bout of thrombotic thrombocytopenic purpura (TTP) developing after adolescence or during adulthood...
October 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29018042/gwas-on-prolonged-gestation-post-term-birth-analysis-of-successive-finnish-birth-cohorts
#12
William Schierding, Jisha Antony, Ville Karhunen, Marja Vääräsmäki, Steve Franks, Paul Elliott, Eero Kajantie, Sylvain Sebert, Alex Blakemore, Julia A Horsfield, Marjo-Riitta Järvelin, Justin M O'Sullivan, Wayne S Cutfield
BACKGROUND: Gestation is a crucial timepoint in human development. Deviation from a term gestational age correlates with both acute and long-term adverse health effects for the child. Both being born preterm and post-term, that is, having short and long gestational ages, are heritable and influenced by the prenatal and perinatal environment. Despite the obvious heritable component, specific genetic influences underlying differences in gestational age are poorly understood. METHODS: We investigated the genetic architecture of gestational age in 9141 individuals, including 1167 born post-term, across two Northern Finland cohorts born in 1966 or 1986...
October 10, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28981198/amplified-endogenous-plasmin-activity-resolves-acute-thrombotic-thrombocytopenic-purpura-in-mice
#13
C Tersteeg, B S Joly, A Gils, R Lijnen, H Deckmyn, P J Declerck, B Plaimauer, P Coppo, A Veyradier, C Maas, S F De Meyer, K Vanhoorelbeke
Essentials Plasmin is able to proteolyse von Willebrand factor. It was unclear if plasmin influences acute thrombotic thrombocytopenic purpura (TTP). Plasmin levels are increased during acute TTP though suppressed via plasmin(ogen) inhibitors. Allowing amplified endogenous plasmin activity in mice results in resolution of TTP signs. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening pathology, caused by occlusive von Willebrand factor (VWF)-rich microthrombi that accumulate in the absence of ADAMTS-13...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28980147/adamts13-missense-variants-associated-with-defective-activity-and-secretion-of-adamts13-in-a-patient-with-non-cirrhotic-portal-hypertension
#14
Ashish Goel, V Raghupathy, G J Amirtharaj, Aaron Chapla, Aparna Venkatraman, Banumathi Ramakrishna, Anup Ramachandran, Nihal Thomas, K A Balasubramanian, Ian Mackie, Elwyn Elias, Chundamannil E Eapen
BACKGROUND: Non-cirrhotic intrahepatic portal hypertension (NCIPH) is characterized by thrombotic microangiopathy of the portal venous system, low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs-13), and high vWF (von Willebrand factor) levels. This study aimed to screen for ADAMTS13 mutations, focusing on the CUB domain, in these patients. METHODS: Prospectively recruited NCIPH patients and healthy volunteers underwent tests for plasma vWF-ADAMTS13 balance...
October 5, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28978852/new-developments-in-treatment-modalities-of-thrombotic-thrombocytopenic-purpura
#15
Masanori Matsumoto
Although thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, appropriate diagnosis and treatment result in the higher survival rate of >80%. TTP is usually suspected with thrombocytopenia and hemolytic anemia and is confirmed by a reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) <10%. TTP is classified as acquired if a patient tests positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are identified...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28940604/asfa-category-iv-becomes-category-i-idiopathic-thrombotic-thrombocytopenic-purpura-in-a-patient-with-presumed-gemcitabine-induced-thrombotic-microangiopathy
#16
Peter G Bittar, Myles S Nickolich, Oluwatoyosi A Onwuemene
In the implementation of American Society for Apheresis national guidelines, the decision for therapeutic plasma exchange may be confounded by a clinical presentation that fits both a Category I and IV designation. We report the case of a 45-year-old female who presented with concern for a Category IV disorder, gemcitabine-induced thrombotic microangiopathy, and was ultimately diagnosed with a Category I disorder, idiopathic thrombotic thrombocytopenic purpura. This case highlights the importance of ruling out idiopathic TTP by a thorough evaluation for ADAMTS13 activity and inhibitor, even when an alternate thrombotic microangiopathy diagnosis may be likely...
September 23, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28940540/successful-kidney-transplantation-in-a-patient-with-congenital-thrombotic-thrombocytopenic-purpura-upshaw-schulman-syndrome
#17
Hasan Fattah, Dhiren Kumar, James N George, H Davis Massey, Anne L King, Kenneth D Friedman, Gaurav Gupta
BACKGROUND: Congenital thrombotic thrombocytopenic purpura (TTP) may not be recognized until organ failure related to the microvascular thrombosis occurs. Kidney failure may be the initial presenting clinical feature. Kidney transplantation has been contraindicated because of the assumption that the continuing microvascular thrombosis will cause inevitable graft failure. CASE REPORT: We report a 48-year-old nulliparous woman who presented with end-stage kidney disease that was attributed to hypertension...
September 20, 2017: Transfusion
https://www.readbyqxmd.com/read/28939980/targeted-exome-sequencing-in-anti-factor-h-antibody-negative-hus-reveals-multiple-variations
#18
R W Thergaonkar, Ankita Narang, Bahadur Singh Gurjar, Pradeep Tiwari, Mamta Puraswani, Himanshi Saini, Aditi Sinha, Binuja Varma, Mitali Mukerji, Pankaj Hari, Arvind Bagga
BACKGROUND: Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation. METHODS: We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic variations in a panel of 15 genes, i.e., CFH, CFHR1-5, CFI, CFB, C3, CD46, MASP2, DGKE, ADAMTS13, THBD and PLG using next-generation DNA sequencing and for copy number variation in CFHR1-3...
September 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28934202/platelet-dysfunction-contributes-to-bleeding-complications-in-patients-with-probable-leptospirosis
#19
Rahajeng N Tunjungputri, Muhammad Hussein Gasem, Willemijn van der Does, Pandu H Sasongko, Bambang Isbandrio, Rolf T Urbanus, Philip G de Groot, Andre van der Ven, Quirijn de Mast
BACKGROUND: Severe leptospirosis is frequently complicated by a hemorrhagic diathesis, of which the pathogenesis is still largely unknown. Thrombocytopenia is common, but often not to the degree that spontaneous bleeding is expected. We hypothesized that the hemorrhagic complications are not only related to thrombocytopenia, but also to platelet dysfunction, and that increased binding of von Willebrand factor (VWF) to platelets is involved in both platelet dysfunction and increased platelet clearance...
September 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28894574/decreased-activity-of-plasma-adamts13-are-related-to-enhanced-cytokinemia-and-endotoxemia-in-patients-with-acute-liver-failure
#20
Hiroaki Takaya, Hitoshi Yoshiji, Hideto Kawaratani, Kazuya Sakai, Masanori Matsumoto, Yoshihiro Fujimura, Hiroshi Fukui
Deficient ADAM metalloproteinase with thrombospondin type-1 motif, member 13 (ADAMTS13) activity (ADAMTS13:AC) results in the accumulation of unusually large von Willebrand factor multimers (UL-VWFM) and causes microcirculatory disturbances and multiple organ failure, while endotoxins trigger the activation of a coagulation cascade. The objective of the present study was to explore the role of ADAMTS13 in endotoxemia in patients with acute liver failure (ALF). Plasma concentrations of endotoxin and cytokines, including interleukin (IL)-6 and IL-8, and activity of the plasma ADAMTS13 inhibitor were determined, along with ADAMTS13:AC, the VWF antigen (VWF:Ag) and UL-VWFM, in 27 patients with acute hepatitis (AH), 11 patients with ALF, and 10 healthy controls...
September 2017: Biomedical Reports
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