Haemophilia

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Effect of recombinant factor VIIa variant (NN1731) on platelet function, clot structure and force onset time in whole blood from healthy volunteers and haemophilia patients
Haemophilia - Tập 13 Số 5 - Trang 533-541 - 2007
Donald F. Brophy, Erika G. Martin, Melinda E. Nolte, Janice Kuhn, Marcus E. Carr
Summary.  NN1731 is a novel variant of recombinant factor VIIa (rFVIIa) that binds to activated platelets, but has greater enzymatic activity than rFVIIa in generating FXa and thrombin. The effect of NN1731 on clot structure and platelet function was characterized ex vivo in whole blood from healthy volunteers and haemophilic patients. Bloo...... hiện toàn bộ
Enhancement of factor VIIa haemostatic efficacy by formulation with PEGylated liposomes
Haemophilia - Tập 14 Số 3 - Trang 476-483 - 2008
Rivka Yatuv, I. DAYAN, Lea Carmel-Goren, Micah Robinson, Irit Aviv, Mirela Goldenberg-Furmanov, Moshe Baru
Summary.  Recombinant activated factor VII (rFVIIa) is an effective treatment of the haemophilia patient with inhibitors and acquired haemophilia. However, on account of its relatively short half‐life (HL), achieving therapeutic efficacy with FVIIa requires repeated injections. The development of a long‐acting FVIIa product would therefore be beneficial. The formulat...... hiện toàn bộ
Effects of factor VIII inhibitor bypassing activity (FEIBA), recombinant factor VIIa or both on thrombin generation in normal and haemophilia A plasma
Haemophilia - Tập 14 Số 4 - Trang 782-786 - 2008
Gili Kenet, Uri Martinowitz, Ariella Zivelin, Uri Seligsohn
Summary.  Factor VIII inhibitor bypass activity (FEIBA) and recombinant factor VIIa (rFVIIa) are the common bypassing agents for treating haemophilia A or haemophilia B patients who developed an inhibitor to factor VIII or IX, respectively. As these preparations differ in their composition and mode of action, combined therapy, either sequential or simultaneous has re...... hiện toàn bộ
Rationale for the use of high dose rFVIIa in a high‐titre inhibitor patient with haemophilia B during major orthopaedic procedures
Haemophilia - Tập 7 Số 5 - Trang 517-522 - 2001
Herbert Cooper, Connie P. Jones, Edmund Campion, Barbara A. Konkle, Ulla Hedner
Inhibitor development is a serious complication in patients with haemophilia A and B. Historically, a lack of optimal therapies and factor products for treating inhibitor patients resulted in many patients developing chronic haemophilic arthropathies and flexion contractures of the involved joints. The introduction of immune‐tolerance protocols to eradicate high‐titre inhibitors has greatl...... hiện toàn bộ
Coronary artery calcification score and carotid intima media thickness in patients with von Willebrand disease
Haemophilia - Tập 19 Số 3 - 2013
M. Zwiers, Joop D. Lefrandt, Douwe J. Mulder, Andries J. Smit, Rijk O. B. Gans, Rozemarijn Vliegenthart, A. V. M. Brands‐Nijenhuis, Hanneke C. Kluin‐Nelemans, Karina Meijer
Results of a phase I/II open‐label, safety and efficacy trial of coagulation factor IX (recombinant), albumin fusion protein in haemophilia B patients
Haemophilia - Tập 21 Số 6 - Trang 784-790 - 2015
Uri Martinowitz, Toshko Lissitchkov, Aaron Lubetsky, Gantcho Jotov, Tami Brutman‐Barazani, C. Voigt, Iris Jacobs, T. Wuerfel, Elena Santagostino
IntroductionrIX‐FP is a coagulation factor IX (recombinant), albumin fusion protein with more than fivefold half‐life prolongation over other standard factor IX (FIX) products available on the market.AimThis prospective phase II, open‐label study evaluated the safety and efficacy of rIX‐FP for...... hiện toàn bộ
In silico calculated affinity of FVIII‐derived peptides for HLA class II alleles predicts inhibitor development in haemophilia A patients with missense mutations in the F8 gene
Haemophilia - Tập 20 Số 2 - Trang 176-184 - 2014
Anastas Pashov, Thierry Calvez, Laurent Gilardin, Bernard Maillère, Yohann Repessé, Johannes Oldenburg, A. Pavlova, Srini V. Kaveri, Sébastien Lacroix‐Desmazes
SummaryForty per cent of haemophilia A (HA) patients have missense mutations in the F8 gene. Yet, all patients with identical mutations are not at the same risk of developing factor VIII (FVIII) inhibitors. In severe HA patients, human leucocy...... hiện toàn bộ
Prolonged effect of a new O-glycoPEGylated FVIII (N8-GP) in a murine saphenous vein bleeding model
Haemophilia - Tập 19 Số 6 - Trang 913-919 - 2013
A. E. Pastoft, Mirella Ezban, Mikael Tranholm, Jens Lykkesfeldt, Brian Lauritzen
Safety procedures of coagulation factors
Haemophilia - Tập 13 Số s5 - Trang 41-46 - 2007
Juan I. Jorquera
Abstract  Two main types of safety procedures must be applied to biological products, including plasma derivatives: (i) preventive procedures and (ii) elimination procedures.Prevention includes epidemiological control of donor populations; checks on each donor’s health condition; analysis of each donation for the main pathogens using serological methods; additional a...... hiện toàn bộ
Nanofiltration of plasma‐derived biopharmaceutical products
Haemophilia - Tập 9 Số 1 - Trang 24-37 - 2003
Thierry Burnouf, Mirjana Radosevich
Summary. This review presents the current status on the use and benefits of viral removal filtration systems – known as nanofiltration – in the manufacture of plasma‐derived coagulation factor concentrates and other biopharmaceutical products from human blood origin.Nanofiltration of plasma products has been implemented at a production scale in the e...... hiện toàn bộ
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