Results 41 to 50 of about 10,178 (166)

Recombinant ADAMTS13 in Congenital Thrombotic Thrombocytopenic Purpura. [PDF]

BACKGROUND Congenital thrombotic thrombocytopenic purpura (TTP) results from severe hereditary deficiency of ADAMTS13. The efficacy and safety of recombinant ADAMTS13 and standard therapy (plasma-derived products) administered as routine prophylaxis ...
Xiao, Shan, Cataland, Spero R, Kremer Hovinga, Johanna A, López-Fernández, M Fernanda, Hassenpflug, Wolf-Achim, Zhang, Pinghai, Scully, Marie, Ortel, Thomas L, Coppo, Paul, Dossier, Claire, Bhattacharya, Indranil, Cronin, Michael, Matsumoto, Masanori, Mellgård, Björn, Patwari, Parth, Patel, Munjal, Li, Hong, Biebuyck, Nathalie, Kentouche, Karim, Knöbl, Paul, Antun, Ana, Windyga, Jerzy, Wang, Linda T   +22 more
core   +1 more source

ADAMTS13 in pediatric sepsis: a prognostic biomarker with potential therapeutic implications

Egyptian Pediatric Association Gazette, 2023
Background Growing evidence implicates a pro-thrombotic state, caused by ADAMTS13 deficiency, in sepsis-associated organ dysfunction, but pediatric data is limited.
Muhammad Said El-Mekkawy, Sara Mahmoud El-Deeb, Maha Fawzy El-Hanafy   +2 more
doaj   +1 more source

Perinatal outcomes in normotensive versus hypertensive HELLP syndrome

International Journal of Gynecology &Obstetrics, EarlyView.
Abstract Objective To compare maternal and neonatal outcomes between women with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome with and without associated hypertension. Methods This was a retrospective cohort study at a tertiary university‐affiliated medical center including all singleton HELLP cases >24 weeks gestation (January ...
Matan Anteby, Keren‐Or Wertheimer, Omri Dominsky, Liran Hiersch, Yariv Yogev   +4 more
wiley   +1 more source

Untersuchungen über ADAMTS13 in Gerinnungsfaktorpräparaten [PDF]

, 2011
Zusammenfassung rnIn der vorliegenden Arbeit wurden sechs VWF/FVIII Gerinnungsfaktorkonzentrate unterschiedlicher Chargen auf ihre ADAMTS13 Aktivität, Antigen und VWF Multimere untersucht.
Falter, Tanja
core   +1 more source

Development of a protease-resistant ADAMTS13 to improve stability against proteolytic degradation

Blood Advances
: Recombinant ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was recently approved by the US Food and Drug Administration for the treatment of heritable thrombotic thrombocytopenic purpura, and preclinical ...
Veronica DeYoung, Rex Huang, Hasam Madarati, Rida Malik, Peter Andrisani, Cherie Teney, Colin A. Kretz   +6 more
doaj   +1 more source

A conformation-sensitive monoclonal antibody against the A2 domain of von Willebrand factor reduces its proteolysis by ADAMTS13. [PDF]

PLoS ONE, 2011
The size of von Willebrand factor (VWF), controlled by ADAMTS13-dependent proteolysis, is associated with its hemostatic activity. Many factors regulate ADAMTS13-dependent VWF proteolysis through their interaction with VWF.
Jingyu Zhang, Zhenni Ma, Ningzheng Dong, Fang Liu, Jian Su, Yiming Zhao, Fei Shen, Anyou Wang, Changgeng Ruan   +8 more
doaj   +1 more source

Deficiency of high‐molecular‐weight von Willebrand factor mitigates thrombo‐complement injury in an LPS‐induced TMA‐like mouse model

VIEW, EarlyView.
Reduction of high‐molecular‐weight von Willebrand factor disrupts the platelet–complement amplification loop, attenuating microvascular thrombosis, complement deposition, endothelial injury, and organ damage in complement‐mediated thrombotic microangiopathy.
Yang Li, Chihao Zhang, Xiaoqian Xu, Sicong Wang, Xiaofan Hu, Junjie Yao, Xi Wu, Difeng Zhu, Qian Liang, Yeling Lu, Yu Liu, Guanqun Xu, Jiakuan Zhou, Qiulan Ding, Wenman Wu, Ya Li, Jing Dai, Xuefeng Wang   +17 more
wiley   +1 more source

Thrombotic Microangiopathy‐Like Phenotype in Patients With Infection‐Associated Disseminated Intravascular Coagulation Treated With Thrombomodulin Alfa

European Journal of Haematology, EarlyView.
ABSTRACT Despite disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) sharing features of thrombocytopenia, organ dysfunction, and bleeding, the relationship between these two conditions remains unclear. We therefore conducted a post hoc analysis of post‐marketing surveillance data from Japan to evaluate the clinical ...
Naoki Takezako, Fumiyo Komatsu, Goichi Honda, Noriaki Kawano, Toshimasa Uchiyama, Kazuo Kawasugi, Seiji Madoiwa, Kei Suzuki, Yoshinobu Seki, Takayuki Ikezoe, Kohji Okamoto, Hideo Wada   +11 more
wiley   +1 more source

Von Willebrand Factor and ADAMTS13 in COVID-19 and Beyond: A Question of Balance

European Medical Journal Nephrology, 2021
von Willebrand factor (VWF) is a large, adhesive, multimeric protein involved in haemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect in VWF can lead to von Willebrand disease
Emmanuel J Favaloro, Brandon Michael Henry, Giuseppe Lippi   +2 more
doaj  

Von Willebrand Factor and ADAMTS13 in Relation to Atherosclerosis in the General Population: The Rotterdam Study

European Journal of Haematology, EarlyView.
ABSTRACT Background Higher von Willebrand factor (VWF) levels and lower ADAMTS13 activity are linked to increased risk of atherosclerotic cardiovascular disease, but their association with atherosclerosis burden, a potential underlying mechanism, remains unclear.
Mitra Nekouei Shahraki, Frank W. G. Leebeek, Moniek P. M. de Maat, Maryam Kavousi, Daniel Bos   +4 more
wiley   +1 more source