Results 41 to 50 of about 12,902 (159)

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

Anti‐ADAMTS13 Antibodies Trajectory is Associated With ADAMTS13 Recovery in Immune‐Mediated TTP

American Journal of Hematology, EarlyView.
Anti‐ADAMTS13 IgG antibodies titer trajectory from baseline to day 7–14 post‐TPE as a reliable approach to identify iTTP patients at risk of late response to the triplet therapy regimen. ABSTRACT Current triplet regimens associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab have ...
Marie Robert, Arthur Mageau, Ygal Benhamou, François Provôt, Jehane Fadlallah, Lionel Galicier, Elie Azoulay, Hafid Ait‐Oufella, Tomas Urbina, Pascale Poullin, Alain Wynckel, Coralie Poulain, Nihal Martis, Pierre Perez, Virginie Rieu, Yahsou Delmas, Jean‐Michel Halimi, Christelle Barbet, Amélie Seguin, Valérie Chatelet, Jean‐François Augusto, Mathieu Legendre, Olivier Moranne, Carole Philipponnet, Bérengère Cador, Raïda Bouzid, Bérangère S. Joly, Agnès Veyradier, Paul Coppo, on behalf of the French Reference Center for Thrombotic Microangiopathies   +29 more
wiley   +1 more source

Diagnosis and follow‐up of thrombotic thrombocytopenic purpura with an automated chemiluminescent ADAMTS13 activity immunoassay

Research and Practice in Thrombosis and Haemostasis, 2021
Background Thrombotic thrombocytopenic purpura (TTP) is a life‐threatening thrombotic microangiopathy (TMA) caused by a severe functional deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I repeats‐13), the specific von ...
Nicolas Beranger, Sandrine Benghezal, Bérangère S. Joly, Sophie Capdenat, Adeline Delton, Alain Stepanian, Paul Coppo, Agnès Veyradier   +7 more
doaj   +1 more source

Elevated plasma neutrophil elastase concentration is associated with disease activity in patients with thrombotic thrombocytopenic purpura. [PDF]

, 2015
INTRODUCTION: Genetic and autoimmune risk factors contribute to the development of thrombotic thrombocytopenic purpura (TTP) but triggers are needed to bring about acute disease.
Brill, Brinkmann, Brinkmann, Bálint Mikes, Chapman, Chaturvedi, Chen, Clark, Clark, Derzsy, Dorottya Csuka, Falter, Fitzpatrick, Fuchs, Fujimura, Gombos, Gyula Domján, György Sinkovits, Hung, Ishikawa, Jagels, Judit Demeter, Karpman, Katalin Rázsó, Kessenbrock, Kremer Hovinga, Leffler, Marienn Réti, Massberg, Moatti-Cohen, Noris, Papayannopoulos, Peng, Peyvandi, Péter Farkas, Raife, Reti, Tsai, Urban, van Montfoort, Walters, Westwood1, Xu, Zoltán Prohászka, Ágota Schlammadinger   +44 more
core   +1 more source

Long noncoding RNA GCH1 mediates mitophagy via the PTEN‐induced kinase 1/Parkin pathway to drive chondrocyte dysfunction and cartilage degeneration in osteoarthritis

Animal Models and Experimental Medicine, EarlyView.
The graphical abstract illustrates the role of lncRNA‐GCH1 in regulating mitophagy and its downstream effects on chondrocyte dysfunction and cartilage degradation in osteoarthritis (OA). Elevated lncRNA‐GCH1 stabilizes PINK1 protein, enhancing mitophagy and mitochondrial turnover.
Gang Zeng, Yujun Sun, Taihe Liu, Wenzhou Liu, Yanbo Chen, Jionglin Wu, Jiayuan Zheng, Weidong Song, Yue Ding   +8 more
wiley   +1 more source

Generation and validation of small ADAMTS13 fragments for epitope mapping of anti‐ADAMTS13 autoantibodies in immune‐mediated thrombotic thrombocytopenic purpura

Research and Practice in Thrombosis and Haemostasis, 2020
Background In immune‐mediated thrombotic thrombocytopenic purpura (iTTP), patients develop an immune response against the multidomain enzyme ADAMTS13.
Kadri Kangro, Elien Roose, An‐Sofie Schelpe, Edwige Tellier, Gilles Kaplanski, Jan Voorberg, Simon F. De Meyer, Andres Männik, Karen Vanhoorelbeke   +8 more
doaj   +1 more source

Signalment risk factors for cutaneous and renal glomerular vasculopathy (Alabama rot) in dogs in the UK [PDF]

, 2018
Seasonal outbreaks of cutaneous and renal glomerular vasculopathy (CRGV) have been reported annually in UK dogs since 2012, yet the aetiology of the disease remains unknown. The objectives of this study were to explore whether any breeds had an increased
Cardwell, J M, Holm, L P, Jepson, R E, O'Neill, D G, Stevens, K B, Walker, D J   +5 more
core   +1 more source

Use of PopPK and E‐R Analyses toward Explaining Causal Link Between ADAMTS13 in Recombinant vs. Plasma‐Based Therapies and Clinical Effects in cTTP

Clinical Pharmacology &Therapeutics, EarlyView.
Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra‐rare, potentially life‐threatening condition caused by a deficiency of the blood enzyme ADAMTS13. Until now, ADAMTS13 replacement has been achieved with infusions of plasma or plasma‐based therapies (PBT).
Munjal Patel, Huijuan Xu, Olivier Barriere, Paul Diderichsen, Parth Patwari, Andy Z. X. Zhu, Jean François Marier, Thomas Peyret, Linda T. Wang, Björn Mellgård, Wenping Wang, Indranil Bhattacharya   +11 more
wiley   +1 more source

ADAMTS13 activity decreases in the early phase of trauma associated with coagulopathy and systemic inflammation: a prospective observational study

Thrombosis Journal, 2021
Background We conducted a prospective observational study for investigating the changes in the 13th member of a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS13) and its association with the coagulofibrinolytic response in ...
Hironori Matsumoto, Jun Takeba, Kensuke Umakoshi, Satoshi Kikuchi, Muneaki Ohshita, Suguru Annen, Naoki Moriyama, Yuki Nakabayashi, Norio Sato, Mayuki Aibiki   +9 more
doaj   +1 more source

Application of PLASMIC Score in Risk Prediction of Thrombotic Thrombocytopenic Purpura: Real-World Experience From a Tertiary Medical Center in Taiwan

Frontiers in Medicine, 2022
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency (activity <10%).
Chun-Hui Lee, Chun-Hui Lee, Chun-Hui Lee, Yi-Ching Huang, Yi-Ching Huang, Sin-Syue Li, Sin-Syue Li, Ya-Ting Hsu, Ya-Ting Hsu, Ya-Ping Chen, Tsai-Yun Chen, Tsai-Yun Chen   +11 more
doaj   +1 more source