Results 31 to 40 of about 10,178 (166)

Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease. [PDF]

PLoS ONE, 2017
Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is known about a ...
Frederik Denorme, Peter Kraft, Inge Pareyn, Christiane Drechsler, Hans Deckmyn, Karen Vanhoorelbeke, Christoph Kleinschnitz, Simon F De Meyer   +7 more
doaj   +1 more source

Novel ADAMTS13 mutations in an obstetric patient with upshaw-schulman syndrome [PDF]

, 2013
Upshaw-Schulman syndrome (USS) is a rarely reported congenital form of thrombotic thrombocytopenic purpura (TTP) that results from mutations in the ADAMTS13 gene.
Johanna A. Kremer Hovinga, Jill Adamski, Taylor Deal, Deal, Taylor, Marisa B. Marques, Kremer Hovinga, Johanna A., Marques, Marisa B., Adamski, Jill   +7 more
core   +1 more source

Anti-ADAMTS13 Antibodies and a Novel Heterozygous p.R1177Q Mutation in a Case of Pregnancy-Onset Immune-Mediated Thrombotic Thrombocytopenic Purpura

TH Open, 2018
In this study, we investigated a case of pregnancy-onset thrombotic thrombocytopenic purpura (TTP). The patient had severely decreased ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13) activity levels during acute ...
Elien Roose, Claudia Tersteeg, Ruth Demeersseman, An-Sofie Schelpe, Louis Deforche, Inge Pareyn, Aline Vandenbulcke, Nele Vandeputte, Daan Dierickx, Jan Voorberg, Hans Deckmyn, Simon F. De Meyer, Karen Vanhoorelbeke   +12 more
doaj   +1 more source

Novel cryptic ADAMTS13 epitopes uncover a distinct open ADAMTS13 conformation in immune-mediated TTP

Haematologica
Open ADAMTS13 conformation is gaining clinical interest as a biomarker for diagnosing immune-mediated thrombotic thrombocytopenic purpura (iTTP) and monitoring remission patients for increased relapse risks.
Quintijn Bonnez, Febe Boudry, Laure De Waele, Lisa Vermeersch, Kadri Kangro, Inge Pareyn, Edwige Tellier, Gilles Kaplanski, Claudia Tersteeg, Bérangère S. Joly, Paul Coppo, Agnès Veyradier, Simon F. De Meyer, Karen Vanhoorelbeke   +13 more
doaj   +1 more source

Cerebral Small Vessel Disease in Immune‐Mediated Thrombotic Thrombocytopenic Purpura Patients During the Acute Phase and Disease Remission

American Journal of Hematology, EarlyView.
ABSTRACT Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a life‐threatening thrombotic microangiopathy characterized by acute neurological manifestations and long‐term sequelae related to microvascular brain injury. Cerebral small vessel disease (cSVD), detectable on MRI through lacunes, microbleeds, white matter hyperintensities, and ...
Addolorata Truma, Francesco Maria Lo Russo, Giorgio Conte, Ilaria Mancini, Andrea Artoni, Juri Alessandro Giannotta, Barbara Ferrari, Pasquale Agosti, Maria Abbattista, Matteo Gagliardi, Eleonora Piccin, Marco Stroppi, Fabio Maria Triulzi, Flora Peyvandi   +13 more
wiley   +1 more source

The relationship between ADAMTS13 genotype and phenotype in congenital thrombotic thrombocytopenic purpura and characterisation of ADAMTS13 mutants [PDF]

, 2015
Congenital thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, usually involving ADAMTS13 gene defects. ADAMTS13 processes the multimeric plasma glycoprotein Von Willebrand factor making it less reactive to platelets.
Underwood, MI
core  

A phenotype–genotype correlation of ADAMTS13 mutations in congenital thrombotic thrombocytopenic purpura patients treated in the United Kingdom

, 2012
Background:  ADAMTS13 mutations play a role in thrombotic thrombocytopenic purpura (TTP) pathogenesis. Objectives:  To establish a phenotype–genotype correlation in a cohort of congenital TTP patients.
Machin, S.J., Camilleri, R., Scully, M., Manns, K., Thomas, M., Mackie, I.J., Chen, W., Liesner, R.   +7 more
core   +1 more source

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome

Haematologica, 2011
Background The assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear.Design and ...
Shangbin Yang, Ming Jin, Shili Lin, Spero Cataland, Haifeng Wu   +4 more
doaj   +1 more source

Von Willebrand Factor, ADAMTS13 and D-Dimer Are Correlated with Different Levels of Nephropathy in Type 1 Diabetes Mellitus. [PDF]

PLoS ONE, 2015
We have investigated whether von Willebrand factor, ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13), and D-Dimer were associated with different levels of renal function in patients with type 1 diabetes. Patients
Caroline Pereira Domingueti, Luci Maria S Dusse, Rodrigo Bastos Fóscolo, Janice Sepúlveda Reis, Joyce Maria Annichino-Bizzacchi, Fernanda Loureiro de Andrade Orsi, Bruna de Moraes Mazetto, Maria das Graças Carvalho, Karina Braga Gomes, Ana Paula Fernandes   +9 more
doaj   +1 more source

ADAMTS13 activity in sickle cell disease

, 2006
Sickle red blood cell (SRBC)-endothelial adhesion plays a central role in sickle cell disease (SCD)-related vaso-occlusion. As unusually large von Willebrand factor (ULVWF) multimers mediate SRBC-endothelial adhesion, we investigated the activity of ...
Krieg, S, Mac Gillavry, MR, Duits, AJ, Hovinga, JA, Akin, S, CURAMA, Study Group, Lämmle, B, Schnog, JJ, Brandjes, DP, Muskiet, FD   +9 more
core   +1 more source