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https://www.readbyqxmd.com/read/27920819/co-administration-of-metformin-and-n-acetylcysteine-with-dietary-control-improves-the-biochemical-and-histological-manifestations-in-rats-with-non-alcoholic-fatty-liver
#1
Naglaa Mohamed El-Lakkany, Sayed Hassan Seif El-Din, Abdel-Nasser Abdel-Aal Sabra, Olfat Ali Hammam, Fatma Abdel-Latif Ebeid
Non-alcoholic fatty liver disease (NAFLD) is a burgeoning health problem that affects 1/3 of the adult population and an increasing number of children in developed countries. Oxidative stress and insulin resistance are the mechanisms that seem to be mostly involved in its pathogenesis. This study was conceived in a NAFLD rat model to evaluate the efficacy of both metformin (MTF) and N-acetylcysteine (NAC) with dietary control on biochemical and histologic liver manifestations. Rats were classified into nine groups; normal (I), NAFLD-induced by feeding high-fat diet (HFD; II) for 12 weeks, NAFLD switched to regular diet (RD; III), NAFLD-HFD or -RD treated with MTF in a dose of 150 mg/kg (IV, V), NAC in a dose of 500 mg/kg (VI, VII) or MTF+NAC (VIII, IX) respectively for 8 weeks...
October 2016: Research in Pharmaceutical Sciences
https://www.readbyqxmd.com/read/27920236/the-efficacy-of-recombinant-fviii-low-dose-prophylaxis-in-chinese-pediatric-patients-with-severe-hemophilia-a-a-retrospective-analysis-from-the-recare-study
#2
Wanru Yao, Juan Xiao, Xiaoling Cheng, Guoshuang Feng, Changgang Li, Xinsheng Zhang, Qun Hu, Weiqun Xu, Jing Sun, Renchi Yang, Xiaojing Li, Rongfu Zhou, Shimei Lian, Jian Gu, Junde Wu, Yongqiang Zhao, Runhui Wu
OBJECTIVE: This study explores the efficacy of recombinant factor VIII (rFVIII) low-dose prophylaxis in Chinese pediatric patients with severe hemophilia A from the Retrospective Study in Chinese Pediatric Hemophilia A Patients With rFVIII Contained Regular Prophylaxis (ReCARE) population. METHODS: This is additional analysis of the multicenter, retrospective ReCARE study, in which the annual bleeding rate (ABR), annual joint bleeding rate (AJBR), and safety of >12-week, low dose (10-30 IU/kg/wk) rFVIII prophylaxis divided into primary, secondary, and tertiary groups based on the joint status and joint bleeding history were analyzed...
December 5, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27919671/supracondylar-femoral-osteotomy-and-knee-joint-replacement-during-the-same-surgical-procedure-in-a-type-a-haemophiliac-patient-with-knee-flexion-deformity-and-ankylosis
#3
Jose Luis Osma Rueda, Alejandra Oliveros Vargas, Cristian David Sosa
BACKGROUND: Haemophilia A is the cause of diverse musculoskeletal disorders such as ankylosis, arthritis and associated angular deformity. There are few reported cases in patients with haemophilia A in which simultaneous supracondylar femoral osteotomy and knee joint replacement has been performed to treat knee angular deformity and ankylosis. Here we present the case of an 18year old male patient, with an evolution of two years, who was unable to walk due to the presence of an untreated supracondylar fracture in the left femur and ipsilateral haemophilic arthropathy which led him to develop an ankylosis in flexion close to 70°...
December 2, 2016: Knee
https://www.readbyqxmd.com/read/27919309/cost-utility-analysis-of-primary-prophylaxis-compared-with-on-demand-treatment-for-patients-with-severe-hemophilia-type-a-in-colombia
#4
Héctor Eduardo Castro Jaramillo, Mabel Moreno Viscaya, Aurelio E Mejia
OBJECTIVES: This article presents a cost-utility analysis from the Colombian health system perspective comparing primary prophylaxis to on-demand treatment using exogenous clotting factor VIII (FVIII) for patients with severe hemophilia type A. METHODS: We developed a Markov model to estimate expected costs and outcomes (measured as quality-adjusted life-years, QALYs) for each strategy. Transition probabilities were estimated using published studies; utility weights were obtained from a sample of Colombian patients with hemophilia and costs were gathered using local data...
December 6, 2016: International Journal of Technology Assessment in Health Care
https://www.readbyqxmd.com/read/27913545/what-have-we-learned-from-large-population-studies-of-von-willebrand-disease
#5
Robert R Montgomery, Veronica H Flood
Von Willebrand factor (VWF) is a critical regulator of hemostatic processes, including collagen binding, platelet adhesion, and platelet aggregation. It also serves as a carrier protein to normalize plasma factor VIII synthesis, release, and survival. While VWF protein measurements by immunoassay are reasonably comparable between institutions, the measurement of VWF ristocetin cofactor activity (VWF:RCo) has significant variability. Other tests of VWF function, including collagen binding or platelet glycoprotein IIb-IIIa binding, are not universally available, yet these functional defects may cause major bleeding even with normal VWF antigen (VWF:Ag) and VWF:RCo assays...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#6
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913543/hemophilia-and-inhibitors-current-treatment-options-and-potential-new-therapeutic-approaches
#7
Shannon L Meeks, Glaivy Batsuli
The immune response to infused factor concentrates remains a major source of morbidity and mortality in the treatment of patients with hemophilia A and B. This review focuses on current treatment options and novel therapies currently in clinical trials. After a brief review of immune tolerance regimens, the focus of the discussion is on preventing bleeding in patients with hemophilia and inhibitors. Recombinant factor VIIa and activated prothrombin complex concentrates are the mainstays in treating bleeds in patients with inhibitors...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913542/new-therapies-for-hemophilia
#8
Steven W Pipe
Individuals with severe hemophilia have benefitted from 5 decades of advances that have led to widespread availability of safe and efficacious factors VIII and IX, a multidisciplinary integrated care model through a network of specialized hemophilia treatment centers, and aggressive introduction of prophylactic replacement therapy to prevent bleeding and preserve joint health. Yet, there are remaining challenges and treatment gaps which have prevented complete abrogation of all joint bleeding, and progressive joint deterioration may continue in some affected individuals over the course of a lifetime...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913541/what-is-the-role-of-an-extended-half-life-product-in-immune-tolerance-induction-in-a-patient-with-severe-hemophilia-a-and-high-titer-inhibitors
#9
Maissaa Janbain, Steven Pipe
A 10-year-old boy presents with a history of severe hemophilia A and high-titer inhibitor that had failed high-dose immune tolerance induction (ITI) with a recombinant factor VIII (rFVIII) product and a plasma-derived FVIII product. You are asked by his mother whether he should be tried on ITI with an extended half-life product, in particular, consideration of a rFVIIIFc concentrate.
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27906868/individual-clotting-factor-contributions-to-mortality-following-trauma
#10
Ryan C Kunitake, Benjamin M Howard, Lucy Z Kornblith, Sabrinah A Christie, Amanda S Conroy, Mitchell J Cohen, Rachael A Callcut
BACKGROUND: Acute traumatic coagulopathy (ATC) afflicts 20-30% of trauma patients, but the extensive collinearity of the coagulation cascade complicates attempts to clarify global clotting factor dysfunction. This study aims to characterize phenotypes of clotting factor dysfunction and their contributions to mortality after major trauma. METHODS: This prospective cohort study examines all adult trauma patients of the highest activation level presenting to San Francisco General Hospital between 2/2005 and 2/2015...
November 30, 2016: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/27906086/massive-adrenal-vein-aneurysm-mimicking-an-adrenal-tumor-in-a-patient-with-hemophilia-a-a-case-report-and-review-of-the-literature
#11
Richard Sleightholm, Steven Wahlmeier, Jeffrey S Carson, Andjela Drincic, Audrey Lazenby, Jason M Foster
BACKGROUND: Visceral venous aneurysms are exceedingly rare, and until now, there have been no reports of this phenomenon in the adrenal vasculature. This report details the first adrenal venous aneurysm reported in the literature. The aneurysm presented as an 18-cm mass that was initially suspected to be a hematoma or tumor on the basis of the complex medical history of the patient, which included hemophilia A and testicular cancer. After surgical excision, pathologic examination confirmed this mass to be a 15...
December 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27904904/clinical-evaluation-of-glycopegylated-recombinant-fviii-efficacy-and-safety-in-severe-haemophilia-a
#12
Paul Giangrande, Tatiana Andreeva, Pratima Chowdary, Silke Ehrenforth, Hideji Hanabusa, Frank W G Leebeek, Steven R Lentz, Laszlo Nemes, Lone Hvitfeldt Poulsen, Elena Santagostino, Chur Woo You, Wan Hui Ong Clausen, Peter G Jönsson, Johannes Oldenburg
Turoctocog alfa pegol (N8-GP) is a novel glycoPEGylated extended half-life recombinant factor VIII (FVIII) product developed for prophylaxis and treatment of bleeds in patients with haemophilia A, to enable higher activity levels with less frequent injections compared with standard FVIII products. This phase III (NCT01480180), multinational, open-label, non-randomised trial evaluated the safety and clinical efficacy of N8-GP when administered for treatment of bleeds and for prophylaxis, in previously treated patients aged ≥12 years with severe haemophilia A...
December 1, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27904665/curcumin-protects-endothelial-cells-against-homocysteine-induced-injury-through-inhibiting-inflammation
#13
Jian Li, Ming Luo, Nanzi Xie, Jianxin Wang, Li Chen
OBJECTIVE: This study aimed to investigate the protective effects of curcumin on the homocysteine (HCY) induced injury to the endothelial cells. METHODS: Endothelial cells were treated with HCY at different concentrations, and MTT assay was employed to determine an optimal concentration of HCY. Cells were divided into 3 groups: normal control group, HCY group and HCY + curcumin group. In curcumin group, cells were pretreated with 2.5 mmol/L HCY for 2 h and then incubated with curcumin at different concentrations...
2016: American Journal of Translational Research
https://www.readbyqxmd.com/read/27902587/influence-of-factor-viii-level-and-its-inhibitor-titer-on-the-therapeutic-response-to-corticosteroids-alone-in-the-management-of-acquired-hemophilia-a-retrospective-single-center-study
#14
Mathieu Vautier, Hubert de Boysson, Christian Creveuil, Yohan Repesse, Annie Borel-Derlon, Xavier Troussard, Gandhi L Damaj, Boris Bienvenu, Philippe Gautier, Achille Aouba
The treatment of acquired hemophilia (AH) involves discussing whether corticosteroids should be administered alone or combined with immunosuppressant drugs, which increase the risk of infection especially in elderly patients and/or those with autoimmunity or neoplastic diseases, who represent the target population of the disease. Prognostic factors highlighting adequate responses to corticosteroids alone must be identified for satisfactory clinical response and lower infectious risk.We aimed to evaluating the efficacy of corticosteroids alone in the management of AH depending on factor VIII (FVIII, ≥ or <1 IU/dL) levels and/or inhibitor (INH, ≤ or >20 Bethesda units per milliliter [BU/mL]) titer...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27898515/cost-effectiveness-analysis-of-pharmacokinetic-driven-prophylaxis-vs-standard-prophylaxis-in-patients-with-severe-haemophilia-a
#15
Sergio Iannazzo, Paolo A Cortesi, Roberto Crea, Katharina Steinitz, Lorenzo G Mantovani, Alessandro Gringeri
The objective of this study was to assess the cost-effectiveness of pharmacokinetic-driven prophylaxis in severe haemophilia A patients. A microsimulation model was developed to evaluate the cost-effectiveness of pharmacokinetic-driven prophylaxis vs. standard prophylaxis and estimate cost, annual joint bleed rate (AJBR), and incremental cost-effectiveness ratio over a 1-year time horizon for a hypothetical population of 10 000 severe haemophilia A patients. A dose of 30 IU/kg per 48 h was assumed for standard prophylaxis...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27896066/acth-therapy-for-west-syndrome-with-severe-hemophilia-a
#16
Kazuo Kubota, Yoriko Kinomura, Takahiro Yamamoto, Michio Ozeki, Minako Kawamoto, Norio Kawamoto, Toshiyuki Fukao
Hemophilia A is an X-linked recessive disorder caused by factor VIII deficiency, which is an important factor in the coagulation system. Here, we describe a 1-year-old boy with hemophilia A who developed West syndrome (WS). Recombinant factor VIII was administered during adrenocorticotropic hormone (ACTH) therapy to prevent intracranial hemorrhage. Infusion of factor VIII at fixed intervals is useful for the safe administration of ACTH therapy for patients with WS with severe hemophilia A. A coagulation screening test should be performed before ACTH therapy...
2016: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/27894230/factor-viii-delivery-devices-in-haemophilia-a-barriers-and-drivers-for-treatment-adherence
#17
Isabel Fernández-Arias, Hae Kyung Kim
INTRODUCTION AND OBJECTIVE: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). METHOD: A cross-sectional research through a supervised survey and a DCS testing session. RESULTS: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17...
November 1, 2016: Farmacia Hospitalaria
https://www.readbyqxmd.com/read/27894217/diagnosis-clinical-manifestations-and-management-of-rare-bleeding-disorders-in-iran
#18
Akbar Dorgalaleh, Sayed Ezatolla Rafiee Alavi, Shadi Tabibian, Shahrzad Soori, Es'hagh Moradi, Taregh Bamedi, Mansour Asadi, Masumeh Jalalvand, Morteza Shamsizadeh
BACKGROUND: Rare bleeding disorders (RBDs) are heterogeneous disorders, mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate of consanguinity that has a high rate of RBDs. OBJECTIVE: In this study, we present prevalence and clinical presentation as well as management and genetic defects of Iranian patients with RBDs. METHODS: For this study, all relevant publications were searched in Medlin until 2015...
November 28, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27891721/extended-half-life-pegylated-full-length-recombinant-factor-viii-for-prophylaxis-in-children-with-severe-haemophilia-a
#19
E S Mullins, O Stasyshyn, M T Alvarez-Román, D Osman, R Liesner, W Engl, M Sharkhawy, B E Abbuehl
INTRODUCTION: Primary factor VIII (FVIII) prophylaxis is the optimal treatment in children with severe haemophilia A. They are expected to benefit from extended half-life (T1/2 ) FVIII coverage by reduced infusion frequency while maintaining haemostatic efficacy. AIMS: To determine immunogenicity, pharmacokinetics (PK), efficacy, safety and quality of life of prophylaxis with a polyethylene glycol (peg)-ylated FVIII (BAX 855) based on full-length recombinant FVIII (ADVATE) in paediatric previously treated patients (PTPs) with severe haemophilia A...
November 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27885373/a-new-era-of-treatment-for-patients-with-haemophilia-a
#20
Robert Klamroth
Treatment and prevention of bleeding episodes in patients with severe haemophilia A require frequent intravenous injection of factor VIII. Inhibitory antibodies against factor VIII occur in approximately 30 % of these patients during the first exposure days and immune tolerance induction to eradicate the inhibitor is challenging. Prevention of bleeds in patients with haemophilia A and inhibitors is less effective and there is ongoing research for alternative treatment options. A promising approach in 2016 is the development of emicizumab (ACE910), a bi-specific IgG antibody to factor IXa and factor X, that mimics the cofactor function of factor VIII...
November 25, 2016: Hämostaseologie
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