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recombinant factor VII

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https://www.readbyqxmd.com/read/29669438/standardized-management-protocol-in-severe-postpartum-hemorrhage-a-single-center-study
#1
Giuseppe Colucci, Karin Helsing, Franziska Demarmels Biasiutti, Luigi Raio, Pirmin Schmid, Dimitrios A Tsakiris, Balthasar Eberle, Daniel Surbek, Bernhard Lämmle, Lorenzo Alberio
Severe postpartum hemorrhage (sPPH) is an obstetric emergency that needs prompt and effective therapy to reduce the risk of complications. In this study, women who developed sPPH (study cohort, n = 27) were treated according to a standardized management protocol prescribing sequential administration of uterotonic drugs, crystalloids, tranexamic acid, labile blood products, low-dose fibrinogen, and recombinant activated factor VII (rFVIIa). This group was compared to patients treated with different strategies during 2 preceding periods: an in-house guideline regulating the administration of rFVIIa (historical cohort 1, n = 20) and no specific guideline (historical cohort 2, n = 27)...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29618153/activation-of-endoplasmic-reticulum-stress-and-unfolded-protein-response-in-congenital-factor-vii-deficiency
#2
Elisabeth Andersen, Maria Eugenia Chollet, Christiane Filion Myklebust, Mirko Pinotti, Francesco Bernardi, Ampaiwan Chuansumrit, Ellen Skarpen, Per Morten Sandset, Grethe Skretting
Congenital factor (F) VII deficiency is a bleeding disorder caused by a heterogeneous pattern of mutations in the F7 gene. Protein misfolding due to mutations is a strong candidate mechanism to produce the highly represented type I FVII deficiency forms, characterized by a concomitant deficiency of FVII antigen and activity. Misfolded proteins can accumulate within the endoplasmic reticulum (ER) causing ER stress with subsequent activation of the unfolded protein response (UPR). So far, there are limited data on this important issue in FVII deficiency...
April 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29566162/recombinant-activated-factor-vii-is-associated-with-postoperative-thromboembolic-adverse-events-in-bleeding-after-coronary-surgery
#3
Aly Makram Habib, Antonio Maria Calafiore, Marco Cargoni, Massimiliano Foschi, Michele Di Mauro
OBJECTIVES: To evaluate the impact of recombinant activated factor VII (rFVIIa) administration on thromboembolic adverse events (TAEs) in coronary artery bypass grafting (CABG) surgery patients showing postoperative bleeding. METHODS: From January 2004 to May 2015, 180 CABG surgery patients with postoperative bleeding were included in the study. All patients were managed conservatively and 81 (45%) also received rFVIIa. RESULTS: Ten patients developed new TAEs (5...
March 16, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29517971/non-factor-replacement-therapy-for-haemophilia-a-current-update
#4
REVIEW
Massimo Franchini, Pier Mannuccio Mannucci
One of the most challenging issues facing us in the treatment of haemophilia is the development of alloantibodies against infused factor VIII (FVIII) or factor IX (FIX). Inhibitors render factor replacement therapy ineffective, exposing patients to an unacceptably high risk of morbidity and mortality. Besides the well-known bypassing agents (i.e. activated prothrombin complex concentrate and recombinant activated factor VII) used to treat or prevent bleeding in haemophilia patients with inhibitors, there is growing interest in a new class of therapeutic agents which act by enhancing coagulation (i...
February 14, 2018: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29385613/prophylactic-and-therapeutic-interventions-for-bleeding-in-dengue-a-systematic-review
#5
Senaka Rajapakse, Nipun Lakshitha de Silva, Praveen Weeratunga, Chaturaka Rodrigo, Sumadhya Deepika Fernando
The global incidence of dengue has increased sevenfold between 1990 and 2013. Despite a low case fatality rate (<1%), during epidemics, due to the large number of people affected, overall mortality rates can be significant. The risk of clinically significant bleeding in dengue is unpredictable and often contributes to an adverse outcome. This systematic review focuses on the evidence for prophylactic and therapeutic interventions for bleeding in dengue infection. PubMed, CINAHL, Cochrane Library, Embase and Google Scholar were searched for randomized, quasi-randomized and non-randomized, prospective or retrospective studies that had a control group alongside an intervention aimed at stopping or preventing bleeding in dengue infection...
October 1, 2017: Transactions of the Royal Society of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29381944/hemorrhagic-pericardial-effusion-as-the-debut-of-acquired-hemophilia-in-a-chronic-lymphocytic-leukemia-patient-a-case-report-and-a-review-of-acquired-hemophilia-a-related-hematological-malignancies
#6
José María Bastida, María Teresa Cano-Mozo, Felix Lopez-Cadenas, Victor Eduardo Vallejo, Soraya Merchán, Cecilia Santos-Montón, David González-Calle, Javier Carrillo, Ana Africa Martín, Jose Angel Torres-Hernández, Marcos González, Francisco Martín-Herrero, Pedro Pabón, Jose Ramon González-Porras
BACKGROUND: Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII. Spontaneous bleeding symptoms usually affect the skin and muscle, while pericardial effusion is an extremely rare manifestation. In the elderly, anticoagulant treatment is frequent and bleeding symptoms are usually associated with this. CLINICAL FINDINGS: We report a hemorrhagic pericardial effusion as the AHA debut in a patient with untreated chronic lymphocytic leukemia and anticoagulated with apixaban for atrial fibrillation and chronic arterial ischemia...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29369801/prediction-of-human-pharmacokinetics-of-activated-recombinant-factor-vii-and-b-domain-truncated-factor-viii-from-animal-population-pharmacokinetic-models-of-haemophilia
#7
Malte Selch Larsen, Rasmus Vestergaard Juul, Andreas Velsing Groth, Ulrika S H Simonsson, Annemarie T Kristensen, Tom Knudsen, Henrik Agersø, Mads Kreilgaard
Various experimental animal models are used in haemophilia research, however, little is known about how well the different species predict pharmacokinetic (PK) profiles in haemophilia patients. The aim of the current study was to describe the plasma concentration-time profile of recombinant activated factor VII (rFVIIa) and recombinant factor VIII (rFVIII) in several experimental animal models using population PK modelling, and apply a simulation-based approach to evaluate how well the developed animal population PK models predict human PK...
March 30, 2018: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29334511/evaluation-of-recombinant-factor-viia-tranexamic-acid-and-desmopressin-to-reduce-prasugrel-related-bleeding-a-randomised-placebo-controlled-study-in-a-rabbit-model
#8
Fanny Bonhomme, Thomas Lecompte, Charles M Samama, Anne Godier, Pierre Fontana
BACKGROUND: Prasugrel is a thienopyridine that inhibits platelet aggregation more rapidly and effectively than clopidogrel, with an increased bleeding risk. OBJECTIVE: The current study aimed to evaluate the efficacy of three nonspecific haemostatic drugs - recombinant activated factor VII (rFVIIa), tranexamic acid and desmopressin (DDAVP) - to limit blood loss after administration of prasugrel in a rabbit model of bleeding while also evaluating any prothrombotic effects...
March 2018: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/29217234/outcomes-following-three-factor-inactive-prothrombin-complex-concentrate-versus-recombinant-activated-factor-vii-administration-during-cardiac-surgery
#9
Patrick C Harper, Mark M Smith, Nathan J Brinkman, Melissa A Passe, Darrell R Schroeder, Sameh M Said, Gregory A Nuttall, William C Oliver, David W Barbara
OBJECTIVE: To compare outcomes following inactive prothrombin complex concentrate (PCC) or recombinant activated factor VII (rFVIIa) administration during cardiac surgery. DESIGN: Retrospective propensity-matched analysis. SETTING: Academic tertiary-care center. PARTICIPANTS: Patients undergoing cardiac surgery requiring cardiopulmonary bypass who received either rFVIIa or the inactive 3-factor PCC. INTERVENTIONS: Outcomes following intraoperative administration of rFVIIa (263) or factor IX complex (72) as rescue therapy to treat bleeding...
February 2018: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29207137/in-vitro-expression-of-mutant-factor-vii-proteins-and-characterization-of-their-clinical-significance
#10
Amir Mashayekhi, Shirin Shahbazi, Mirdavood Omrani, Reza Mahdian
Factor VII (FVII) serves an essential role in the initiation of blood coagulation. Mutations in conserved residues within its serine protease domain may lead to dysregulated coagulation activity. The objective of the present study was to elucidate the impact of altering two conserved residues, H348R and S282R, on the functional properties of the FVII protein. The mutation‑harboring fragments were derived from genomic DNA of a FVII deficient patient. The fragments were integrated into a pcDNA vector containing FVII cDNA of HepG2 cells...
February 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29204261/recombinant-human-factor-viia-rfviia-in-hemophilia-mode-of-action-and-evidence-to-date
#11
REVIEW
Muriel Giansily-Blaizot, Jean-François Schved
Recombinant activated factor VII (rFVIIa) is a bypassing agent widely used both in the treatment and prevention of hemorrhagic complications due to hemophilia with inhibitor. In such cases, antihemophilic factors cannot be used. The normal physiology of factor VII/ factor VIIa (FVII/FVIIa) in the hemostatic process requires the presence of tissue factor (TF) that links to FVII leading to a FVIIa-TF complex which activates both factor X and factor IX. The therapeutic use of rFVIIa requires high amount of FVIIa...
December 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29132165/ivh-in-vlbw-preterm-babies-therapy-with-recombinant-activated-f-vii
#12
Matthias Knüpfer, Jenny Ritter, Ferdinand Pulzer, Corinna Gebauer, Nadine Wolf, Anett Bläser, Jens Dreyhaupt, Ulrich Thome
Backround Intraventricular hemorrhage (IVH) remains a dangerous and frequent complication in very low birth weight (VLBW) infants. Activated factor VII (aFVII) activates the coagulation cascade and is a potential tool for stopping active bleeding, including limiting the extent of an IVH. This retrospective treatment observation compared data for infants with IVH progression treated with fresh frozen plasma (FFP) alone or with a combination of FFP and aFVII. Methods/Intervention All infants were subject to cranial ultrasonography at least twice daily...
November 2017: Klinische Pädiatrie
https://www.readbyqxmd.com/read/29099345/coagulation-management-during-liver-transplantation-use-of-fibrinogen-concentrate-recombinant-activated-factor-vii-prothrombin-complex-concentrate-and-antifibrinolytics
#13
Jonathan H Chow, Khang Lee, Ezeldeen Abuelkasem, Obi R Udekwu, Kenichi A Tanaka
Coagulation management, and transfusion practice in liver transplantation (LT) have been evolving in the recent years due to better understanding of coagulation abnormalities in end-stage liver disease, and clinical management of LT patients. Avoidance of allogeneic blood components is feasible in some patients, but multi-modal coagulation therapies may be necessary in others who develop complex coagulopathy due to hemorrhage, hemodilution, hypothermia, and acid-base disturbances. Transfusions of plasma and cryoprecipitate remain to be the mainstay therapy for procoagulant factor replacement during LT...
November 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29051804/review-of-recombinant-anti-haemophilic-porcine-sequence-factor-viii-in-adults-with-acquired-haemophilia-a
#14
REVIEW
Emma Fosbury, Anja Drebes, Anne Riddell, Pratima Chowdary
Acquired haemophilia A (AHA) is a rare, serious bleeding disorder most often encountered in elderly patients. The mainstay of haemostatic management is with bypassing agents (BPAs) including recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCCs). Their major limitation is incomplete efficacy, potential risk for thrombosis and the lack of routine laboratory assays for monitoring treatment response. Plasma-derived porcine FVIII (pd-pFVIII, Hyate C® ), first used in the 1950s for the management of congenital haemophilia, has sufficient sequence homology to be haemostatic in humans, but the lack of complete homology facilitates efficacy even in the presence of human allo- and autoantibodies against human FVIII (hFVIII)...
September 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28921441/purification-and-autoactivation-method-for-recombinant-coagulation-factor-vii
#15
Vladimir Granovski, Marcela C C Freitas, Mario Soares Abreu-Neto, Dimas T Covas
Recombinant coagulation factor VII is a very important and complex protein employed for treatment of hemophiliac patients (hemophilia A/B) who develop inhibitors antibodies to conventional treatments (FVIII and FIX). The rFVII is a glycosylated molecule and circulates in plasma as zymogen of 50 kDa. When activated the molecule is cleaved to 20-30 kDa and has a half-life of about 3 h, needing to be processed fast and efficiently until freeze-drying. Here, we describe a very simple and fast purification sequence for rFVII using affinity FVII Select resin and a dialysis system that can be easily scaled up...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28881271/factor-vii-activating-protease-fsap-regulates-the-expression-of-inflammatory-genes-in-vascular-smooth-muscle-and-endothelial-cells
#16
Kristina Byskov, Thomas Boettger, Paul F Ruehle, Nis Valentin Nielsen, Michael Etscheid, Sandip M Kanse
BACKGROUND AND AIMS: The factor VII activating protease (FSAP) knockout mice have a bigger neointima after vascular injury and a larger infarct volume after stroke. The Marburg I (MI) single nucleotide polymorphism (SNP) in the FSAP-encoding gene is associated with an increased risk of stroke and carotid stenosis in humans. We hypothesize that the regulation of gene expression by FSAP in vascular cells accounts for its vasculo-regulatory properties. METHODS: Vascular smooth muscle cells (VSMC) and endothelial cells (EC) were stimulated with FSAP and a microarray-based expression analysis was performed...
August 25, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28767473/acquired-hemophilia-a-after-hepatic-yttrium-90-radioembolization-a-case-report
#17
Susanna Tribuzi, Alessia Naccarato, Lorella Pelagalli, Marco Covotta, Giulia Torregiani, Claudia Claroni, Ester Forastiere
Acquired hemophilia is a rare but potentially life-threatening bleeding disorder caused by the development of autoantibodies (inhibitors) directed against plasma coagulation factors, most frequently factor VIII. We report a case of a 65-year-old man with hepatocellular carcinoma who bled massively after a hepatic Yttrium-90 radioembolization procedure (Selective Internal Radiation Therapy with Yttrium-90 Resin Microspheres [SIRTex]). An acquired deficiency of factor VIII was diagnosed and successfully treated with recombinant activated factor VII and immunosuppression...
December 15, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28686157/the-role-of-recombinant-activated-factor-vii-in-the-haematological-management-of-elective-orthopaedic-surgery-in-haemophilia-a-patients-with-inhibitors
#18
REVIEW
Giancarlo Castaman
The clinical profile and expectations of haemophilic patients with inhibitors have changed over the last three decades, mainly because of the prolongation of life-expectancy, often resulting in an increase of the orthopaedic burden. Recombinant activated factor VII (rFVIIa) is the most frequently used bypassing agent in haemophilia patients with inhibitors during elective orthopaedic surgery. For nearly 30 years, rFVIIa has been successfully used to control haemostasis in several major and minor surgical procedures...
September 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28684918/genotype-and-phenotype-correlation-in-intracranial-hemorrhage-in-neonatal-factor-vii-deficiency-among-thai-children
#19
Chanchai Traivaree, Chalinee Monsereenusorn, Arunotai Meekaewkunchorn, Premsak Laoyookhong, Saranya Suwansingh, Boonchai Boonyawat
Congenital factor VII (FVII) deficiency is a rare inherited coagulopathy. The clinical manifestations and clinical findings vary widely, ranging from asymptomatic to life-threatening bleeding, including intracranial hemorrhage (ICH), with prolonged prothrombin time, normal partial thromboplastin time and normal platelet counts, which are confirmed by the low level of FVII assay. Treatment consists of fresh frozen plasma (FFP), prothrombin complex concentrates (PCCs), and recombinant activated FVII to treat bleeding and prophylactic therapy...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28672139/management-of-surgical-third-lower-molar-extraction-and-postoperative-progress-in-patients-with-factor-vii-deficiency-a-clinical-protocol-and-focus-on-this-rare-pathologic-entity
#20
Pier Carmine Passarelli, Guido Pasquantonio, Antonio D'Addona
PURPOSE: The purpose of the present study was to analyze the management of surgical third molar extraction and postoperative progress in patients with a diagnosis of factor VII deficiency. Close collaboration between the oral-maxillofacial surgeon and hematologist will allow the team to categorize the risk and operate safely, thereby minimizing the incidence and severity of intraoperative and postoperative complications. MATERIALS AND METHODS: The present retrospective study included 7 patients with factor VII deficiency who had undergone third lower molar surgery...
October 2017: Journal of Oral and Maxillofacial Surgery
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