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ADAMTS13

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https://www.readbyqxmd.com/read/28339879/high-frequency-of-mutations-in-genes-encoding-factor-viii-von-willebrand-factor-and-adamts13-in-skin-cutaneous-melanoma
#1
Zheng Ping, Huy P Pham, Lance A Williams, X Long Zhen
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28339859/human-neutrophil-peptides-hnps-inhibit-proteolytic-cleavage-of-von-willebrand-factor-by-adamts13-a-potential-link-between-inflammation-and-thrombotic-thrombocytopenic-purpura
#2
Vikram G Pillai, Jialing Bao, Catherine B Zander, Jenny McDaniel, Steven H Seeholzer, Khalil Bdeir, Douglas B Cines, X Long Zhen
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28326725/-analysis-of-salivary-protease-spectrum-in-chronic-periodontitis
#3
Li Qian, Zhou Xuedong, Fan Yaping, Yang Tengyu, Wu Songtao, Yu Yu, Chen Jiao, Zhang Ping, Feng Yun
OBJECTIVE: This study aimed to investigate the difference in salivary protease expression in patients with chronic periodontitis and normal individuals. METHODS: The stimulating saliva in patients with chronic periodontitis and normal individuals were collected. Protein chip technology was adapted to analyze salivary protease spectrum. RESULTS: Among the 34 proteases in the chip, disintegrin and metalloproteinase (ADAM)8, matrix metalloproteinase (MMP)-8, MMP-12, neprilysin/CD10, and uridylyl phosphate adenosine/urokinase showed a significantly increased concentration in the saliva of chronic periodontitis patients compared with those in the saliva of normal individuals (P<0...
February 1, 2017: Hua Xi Kou Qiang Yi Xue za Zhi, Huaxi Kouqiang Yixue Zazhi, West China Journal of Stomatology
https://www.readbyqxmd.com/read/28321086/acquired-thrombotic-thrombocytopenic-purpura-with-a-smoldering-clinical-course
#4
Chiaki Naito, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Masahiro Mihara, Masaki Hayakawa, Masanori Matsumoto, Yoshihisa Nojima, Hiroshi Handa
Acquired thrombotic thrombocytopenic purpura (aTTP) is caused by a deficiency of ADAMTS13 activity due to neutralizing auto-autoantibodies (inhibitors) against ADAMTS13. Patients with aTTP show a rapid and fatal clinical course, unless an effective therapeutic intervention such as plasma exchange therapy (PEX) is performed. There is, however, a small population of patients who show a smoldering clinical course, for which no effective treatment strategy has yet been established. We herein report a 77-year-old man, who had repeated episodes of cerebral infarction and persistent thrombocytopenia over several months, but was not correctly diagnosed as having aTTP at a local hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#5
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 15, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28283300/microangiopathic-hemolytic-anemia-in-pregnancy
#6
Mohammed Salhab, Andrew Hsu, Elizabeth Ryer, Juliet Appiah, Bradley Switzer
Microangipathic hemolytic anemia (MAHA) is a serious diagnosis and difficult to manage in pregnant patients as multiple life threatening conditions could present with MAHA. ADAMTS13 enzyme activity can be affected during pregnancy with multiple factors. A persistent extremely low ADAMTS13 enzyme activity levels, without an inhibitor, after the delivery was an important factor to establish the diagnosis. We present a case of likely congenital ADAMST13 deficiency that manifested for the first time in a pregnant woman at week 37 of pregnancy...
February 16, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28279966/plasmin-cleaves-von-willebrand-factor-at-k1491-r1492-in-the-a1-a2-linker-region-in-a-shear-and-glycan-dependent-manner-in-vitro
#7
Teresa M Brophy, Soracha E Ward, Thomas R McGimsey, Sonja Schneppenheim, Clive Drakeford, Jamie M O'Sullivan, Alain Chion, Ulrich Budde, James S O'Donnell
OBJECTIVE: Previous studies have demonstrated a role for plasmin in regulating plasma von Willebrand factor (VWF) multimer composition. Moreover, emerging data have shown that plasmin-induced cleavage of VWF is of particular importance in specific pathological states. Interestingly, plasmin has been successfully used as an alternative to ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif) in a mouse model of thrombotic thrombocytopenic purpura. Consequently, elucidating the molecular mechanisms through which plasmin binds and cleaves VWF is not only of basic scientific interest but also of direct clinical importance...
March 9, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28259520/derivation-and-external-validation-of-the-plasmic-score-for-rapid-assessment-of-adults-with-thrombotic-microangiopathies-a-cohort-study
#8
Pavan K Bendapudi, Shelley Hurwitz, Ashley Fry, Marisa B Marques, Stephen W Waldo, Ang Li, Lova Sun, Vivek Upadhyay, Ayad Hamdan, Andrew M Brunner, John M Gansner, Srinivas Viswanathan, Richard M Kaufman, Lynne Uhl, Christopher P Stowell, Walter H Dzik, Robert S Makar
BACKGROUND: Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed. METHODS: All consecutive adult patients presenting to three large academic medical centres in Boston, MA, USA, with thrombotic microangiopathy and a possible diagnosis of thrombotic thrombocytopenic purpura between Jan 8, 2004, and Dec 6, 2015, were included in an ongoing multi-institutional registry (the Harvard TMA Research Collaborative)...
March 1, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28254814/long-term-prevention-of-congenital-thrombotic-thrombocytopenic-purpura-in-adamts13-knockout-mice-by-sleeping-beauty-transposon-mediated-gene-therapy
#9
Sebastien Verhenne, Nele Vandeputte, Inge Pareyn, Zsuzsanna Izsvák, Hanspeter Rottensteiner, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke
OBJECTIVE: Severe deficiency in the von Willebrand factor-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) because of mutations in the ADAMTS13 gene can lead to acute episodes of congenital thrombotic thrombocytopenic purpura (TTP), requiring prompt treatment. Current treatment consists of therapeutic or prophylactic infusions of fresh frozen plasma. However, lifelong treatment with plasma products is a stressful therapy for TTP patients...
March 2, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28249049/platelet-independent-adhesion-of-calcium-loaded-erythrocytes-to-von-willebrand-factor
#10
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage. Adhesion of erythrocytes to vascular endothelial cells has been described in multiple hemolytic disorders, especially in sickle cell disease, but the adhesion of normal erythrocytes to endothelial cells has hardly been described. It was shown that calcium-loaded erythrocytes can adhere to endothelial cells. Because sickle erythrocyte adhesion to ECs can be enhanced by ultra-large von Willebrand factor multimers, we investigated whether calcium loading of erythrocytes could promote binding to endothelial cells via ultra-large von Willebrand factor multimers...
2017: PloS One
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#11
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28209710/a-model-for-the-conformational-activation-of-the-structurally-quiescent-metalloprotease-adamts13-by-von-willebrand-factor
#12
Kieron South, Marta O Freitas, David A Lane
Blood loss is prevented by the multi-domain glycoprotein von Willebrand factor (VWF), which binds exposed collagen at damaged vessels and captures platelets. VWF is regulated by the metalloprotease ADAMTS13, which, in turn, is conformationally activated by VWF. To delineate the structural requirements for VWF-mediated conformational activation of ADAMTS13, we performed binding and functional studies with a panel of truncated ADAMTS13 variants. We demonstrate that both the isolated CUB1 and CUB2 domains in ADAMTS13 bind to the spacer domain exosite of a truncated ADAMTS13 variant, MDTCS (KD of 135 ± 10...
February 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28182324/clumping-factor-a-von-willebrand-factor-binding-protein-and-von-willebrand-factor-anchor-staphylococcus-aureus-to-the-vessel-wall
#13
J Claes, L Liesenborghs, M Peetermans, T R Veloso, D Missiakas, O Schneewind, S Mancini, J M Entenza, M F Hoylaerts, R Heying, P Verhamme, T Vanassche
OBJECTIVE: When establishing endovascular infections, Staphylococcus aureus (S. aureus) overcomes shear forces of flowing blood by binding to von Willebrand factor (VWF). Staphylococcal VWF-binding protein (vWbp) interacts with VWF, but it is unknown how this secreted protein binds to the bacterial cell wall. We hypothesized that vWbp interacts with a staphylococcal surface protein, mediating the adhesion of S. aureus to VWF and vascular endothelium under shear stress. METHODS: We studied the binding of S...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28139813/new-treatment-options-for-thrombotic-thrombocytopenic-purpura
#14
Paul Knöbl
The thrombotic-thrombocytopenic purpura (TTP) is an acute, life-threatening disease, characterised by enhanced platelet aggregation, disturbed microcirculation and organ dysfunction. With the currently available treatment (plasma exchange, infusions, corticosteroids) mortality ist still as high as 10-15 %. Recent, pathophysiology-based developments may improve the outcome. The most promising candidates for future treatment of TTP are: rituximab for termination of the autoimmune process, caplacizumab for prevention of platelet-VWF-interaction, and recombinant ADAMTS13 for replacement of the inhibited or missing enzyme...
November 21, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/28139439/neutrophil-protease-cleavage-of-von-willebrand-factor-in-glomeruli-an-anti-thrombotic-mechanism-in-the-kidney
#15
Ramesh Tati, Ann-Charlotte Kristoffersson, Minola Manea Hedström, Matthias Mörgelin, Jörgen Wieslander, Cees van Kooten, Diana Karpman
Adequate cleavage of von Willebrand factor (VWF) prevents formation of thrombi. ADAMTS13 is the main VWF-cleaving protease and its deficiency results in development of thrombotic microangiopathy. Besides ADAMTS13 other proteases may also possess VWF-cleaving activity, but their physiological importance in preventing thrombus formation is unknown. This study investigated if, and which, proteases could cleave VWF in the glomerulus. The content of the glomerular basement membrane (GBM) was studied as a reflection of processes occurring in the subendothelial glomerular space...
February 2017: EBioMedicine
https://www.readbyqxmd.com/read/28133771/thrombotic-thrombocytopenic-purpura-misdiagnosed-as-autoimmune-cytopenia-causes-of-diagnostic-errors-and-consequence-on-outcome-experience-of-the-french-thrombotic-microangiopathies-reference-centre
#16
Maximilien Grall, Elie Azoulay, Lionel Galicier, François Provôt, Alain Wynckel, Pascale Poullin, Steven Grange, Jean-Michel Halimi, Alexandre Lautrette, Yahsou Delmas, Claire Presne, Mohamed Hamidou, Stéphane Girault, Frédéric Pène, Pierre Perez, Tarik Kanouni, Amélie Seguin, Christiane Mousson, Dominique Chauveau, Mario Ojeda-Uribe, Virginie Barbay, Agnès Veyradier, Paul Coppo, Ygal Benhamou
Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia...
April 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#17
REVIEW
Paul Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
February 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28102428/plasma-adamts13-activity-and-von-willebrand-factor-antigen-and-activity-in-patients-with-subarachnoid-haemorrhage
#18
Monisha Kumar, Wenjing Cao, Jenny K McDaniel, Huy P Pham, Dheeraj Raju, Kelsey Nawalinski, Suzanne Frangos, David Kung, Eric Zager, Scott E Kasner, Joshua M Levine, X Long Zheng
Increased von Willebrand factor (VWF) and reduced ADAMTS13 activity are associated with arterial thrombosis. This may also be the culprit mechanism implicated in delayed cerebral ischaemia after aneurysmal subarachnoid haemorrhage (SAH). It was our objective to determine plasma VWF and ADAMTS13 in patients with SAH and healthy subjects; and to explore the levels of those markers and outcome after SAH. Forty consecutive patients were enrolled between September 2007 and April 2014 in a pilot study. Plasma samples were collected from SAH patients on post-bleed day (PBD) 0, 1, 3, 5, 7 and 10 and healthy controls...
January 19, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28101432/atypical-hemolytic-uremic-syndrome-in-first-trimester-pregnancy-successfully-treated-with-eculizumab
#19
Gabriela Andries, Michael Karass, Srikanth Yandrapalli, Katherine Linder, Delong Liu, John Nelson, Rahul Pawar, Savneek Chugh
BACKGROUND: Atypical hemolytic uremic syndrome is a rare disorder which is known to cause acute thrombotic microangiopathy during pregnancy with poor maternal and fetal outcomes. Atypical hemolytic uremic syndrome is caused mostly by dysregulation of alternative complement pathway secondary to genetic mutations. Most of the cases reported have been in the post-partum period. We report a rare case of a patient who presents with thrombotic microangiopathy in the first trimester of her eleventh pregnancy and was successfully treated with eculizumab...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28090741/conformational-quiescence-of-adamts-13-prevents-proteolytic-promiscuity-reply
#20
LETTER
K South, M O Freitas, D A Lane
No abstract text is available yet for this article.
January 16, 2017: Journal of Thrombosis and Haemostasis: JTH
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