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Factor VIII inhibitor

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https://www.readbyqxmd.com/read/28326849/long-term-efficacy-and-safety-of-prophylaxis-with-rfviii-in-chinese-pediatric-patients-with-hemophilia-a-a-multi-center-retrospective-non-interventional-phase-iv-recare-study
#1
Changgang Li, Xinsheng Zhang, Yongqiang Zhao, Runhui Wu, Qun Hu, Vicky Xu, Jing Sun, Renchi Yang, Xiaojing Li, Rongfu Zhou, Shinmei Lian, Jian Gu, Junde Wu, Qingsong Hou
BACKGROUND: The first recombinant factor VIII (rFVIII) product was launched in China in 2007. However, till date, no study has been conducted to describe the efficacy and safety of prophylaxis with rFVIII in Chinese pediatric patients with hemophilia A (HA). OBJECTIVE: To summarize the efficacy and safety data of prophylaxis with rFVIII in Chinese pediatric patients with HA. METHODS: ReCARE (Retrospective study in Chinese pediatric hemophilia A patients with rFVIII contained regular prophylaxis) was a retrospective study conducted in 12 hemophilia treatment centers (HTC's) across China...
March 22, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28299631/clinical-characteristics-and-outcomes-of-acquired-hemophilia-a-experience-at-a-single-center-in-japan
#2
Yoshiyuki Ogawa, Kunio Yanagisawa, Hideki Uchiumi, Takuma Ishizaki, Takeki Mitsui, Fumito Gouda, Masahiro Ieko, Akitada Ichinose, Yoshihisa Nojima, Hiroshi Handa
Acquired hemophilia A (AHA), which is caused by autoantibodies against coagulation factor VIII (FVIII) is a rare, life-threatening bleeding disorder, the incidence of which appears to be increasing in Japan as the population ages. However, the clinical characteristics, treatment, and outcomes of AHA remain difficult to establish due to the rarity of this disease. We retrospectively analyzed data from 25 patients (median age 73 years; range 24-92 years; male n = 15) diagnosed with AHA between 1999 and 2015 at Gunma University Hospital...
March 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28285015/tnf%C3%AE-contributes-to-diabetes-impaired-angiogenesis-in-fracture-healing
#3
Jason C Lim, Kang I Ko, Marcelo Mattos, Miao Fang, Citong Zhang, Daniel Feinberg, Hisham Sindi, Shuai Li, Jazia Alblowi, Rayyan A Kayal, Thomas A Einhorn, Louis Gerstenfeld, Dana Graves
Diabetes increases the likelihood of fracture, interferes with fracture healing and impairs angiogenesis. The latter may be significant due to the critical nature of angiogenesis in fracture healing. Although it is known that diabetes interferes with angiogenesis the mechanisms remain poorly defined. We examined fracture healing in normoglycemic and streptozotocin-induced diabetic mice and quantified the degree of angiogenesis with antibodies to three different vascular markers, CD34, CD31 and Factor VIII. The role of diabetes-enhanced inflammation was investigated by treatment of the TNFα-specific inhibitor, pegsunercept starting 10days after induction of fractures...
March 8, 2017: Bone
https://www.readbyqxmd.com/read/28276537/recombinant-von-willebrand-factor-a-first-of-its-kind-product-for-von-willebrand-disease
#4
M Singal, P A Kouides
von Willebrand disease (VWD) is caused by quantitative or qualitative defects in von Willebrand factor (VWF). The mainstay of therapy is desmopressin, which is, however, not useful in certain forms of VWD notwithstanding adverse events. For these patients, plasma-derived factor VIII (pdFVIII)/VWF concentrates have been available for close to three decades but have a theoretical risk of disease transmission, hypersensitivity/allergic reactions, inhibitors and thrombosis. A recombinant VWF (vonicog alfa, Vonvendi™; manufactured by Baxalta, now part of Shire) was approved by the U...
December 2016: Drugs of Today
https://www.readbyqxmd.com/read/28262242/an-extra-x-does-not-prevent-acquired-hemophilia-pregnancy-associated-acquired-hemophilia-a
#5
Assaf A Barg, Tami Livnat, Gili Kenet
Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). With an estimated annual incidence of 1.3 to 1.5 per million, AHA is a rare disease. An extremely rare form of AHA has been described among women in the peripartum period, and may present with peripartum hemorrhage. Notably, although hemorrhagic symptoms commonly present 1-4 months around delivery, they may occur up to 1 year after parturition. When caring for a mother with AHA it is important to note that Factor VIII inhibitor may be transferred via the placenta from the mother to the fetus...
March 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28246607/tissue-factor-pathway-inhibitor-1-is-a-valuable-marker-for-the-prediction-of-deep-venous-thrombosis-and-tumor-metastasis-in-patients-with-lung-cancer
#6
Xianming Fei, Huan Wang, Wufeng Yuan, Mingyi Wo, Lei Jiang
Activation of blood coagulation contributes to cancer progression. Tissue factor pathway inhibitor-1 (TFPI-1) is the main inhibitor of extrinsic coagulation pathway. The aim of this study is to assess the predicting significance of TFPI-1 for thrombotic complication and metastasis in lung cancer patients. Total of 188 non-small cell lung cancer (NSCLC) patients were included in this study. Plasma TFPI-1, D-dimer (D-D), antithrombin (AT), Fibrinogen (Fbg), and coagulating factor VIII activity (FVIII:C) were measured...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28243997/safety-and-effectiveness-of-factor-viii-inhibitor-bypassing-activity-feiba-and-fresh-frozen-plasma-in-oral-anticoagulant-associated-intracranial-hemorrhage-a-retrospective-analysis
#7
Ellen B Yin, Benedict Tan, Thuy Nguyen, Miguel Salazar, Kimberly Putney, Pramod Gupta, Jose I Suarez, Eric M Bershad
BACKGROUND: Oral anticoagulant (OAT)-associated intracranial hemorrhage (ICH) is a life-threatening emergency for which prothrombin complex concentrates (PCC) are considered first-line reversal agents. The only approved PCC in the USA for warfarin-associated ICH is non-activated PCC. Little data are available regarding the safety and effectiveness of factor VIII inhibitor bypassing activity (FEIBA) which is an activated prothrombin complex concentrate (aPCC). The aim of this analysis was to assess the safety and effectiveness of FEIBA compared to fresh frozen plasma (FFP) for reversal of OAT-associated ICH...
February 27, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28226363/coagulation-profile-in-patients-with-different-etiologies-for-cushing-syndrome-a-prospective-observational-study
#8
Amit Tirosh, Maya Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Richard A Feelders, Constantine A Stratakis
Previous studies reported a higher prevalence of venous-thromboembolic events among patients with Cushing disease (CD) compared to those with ACTH-independent Cushing syndrome (CS) from adrenal sources. The objective of the current study was to evaluate the coagulation profile of patients with CS from different etiologies. A prospective observational study was conducted at a clinical research center. The study included adult patients admitted for evaluation of suspected CS (n=85), that were divided into 3 groups: CD (n=22), ACTH-independent CS from an adrenal tumor/hyperplasia (adrenal CS, n=21), and a control group consisting of subjects with negative screening for CS (rule-out CS, n=42)...
February 22, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28220685/incidence-of-low-titre-factor-viii-inhibitors-in-patients-with-haemophilia-a-meta-analysis-of-observational-studies
#9
A Messori, F Peyvandi, D Mengato, P M Mannucci
INTRODUCTION: A few studies have been focused on low-titre inhibitors in patients with haemophilia A. Although several putative factors have been implicated in the development of these inhibitors, solid data are still lacking. AIM: The aim of this study was to perform a proportion meta-analysis on the incidence of low-titre inhibitors in haemophilia A. METHODS: We surveyed the PubMed database to identify studies on de novo development of low-titre inhibitors in haemophilia A patients...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28212975/budget-impact-analysis-of-prolonged-half-life-recombinant-fviii-therapy-for-hemophilia-in-the-united-states
#10
Suzanne McMullen, Brieana Buckley, Eric Hall, Jon Kendter, Karissa Johnston
BACKGROUND: Hemophilia A is a factor VIII deficiency, associated with spontaneous, recurrent bleeding episodes. This may lead to comorbidities such as arthropathy and joint replacement, which contribute to morbidity and increased health care expenditure. Recombinant factor VIII Fc fusion protein (rFVIIIFc), a prolonged half-life factor therapy, requires fewer infusions, resulting in reduced treatment burden. OBJECTIVE: Use a budget impact analysis to assess the potential economic impact of introducing rFVIIIFc to a formulary from the perspective of a private payer in the United States...
January 2017: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/28207534/a-case-control-study-on-the-structural-equation-model-of-the-mechanism-of-coagulation-and-fibrinolysis-imbalance-in-chronic-schistosomiasis
#11
Aiping Le, Lunli Zhang, Wei Liu, Xiaopeng Li, Jianwei Ren, An Ning
A structural equation model was used for verification with chronic schistosomiasis to investigate the coagulation-anticoagulation system imbalance and to deduce the mechanism of D-dimer (D-D) level elevation in patients with advanced schistosome hepatic disease. We detected the plasma levels of tissue-type fiber plasminogen activator (tPA), urokinase type plasminogen activator (uPA), plasmin-antiplasmin complex (PAP), plasminogen (PLG), antithrombin (AT), plasminogen activator inhibitor 1 (PAI1), D-D, factor VIII: C (FVIII:C), antithrombin-III (AT-III), PLG, protein S (PS), and protein C (PC) in the healthy people as control (69), patients with chronic schistosomiasis (150) or advanced chronic schistosomiasis (90)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28205285/natural-history-and-clinical-characteristics-of-inhibitors-in-previously-treated-haemophilia-a-patients-a-case-series
#12
A Iorio, A M Barbara, M Makris, K Fischer, G Castaman, C Catarino, E Gilman, K Kavakli, T Lambert, R Lassila, T Lissitchkov, E Mauser-Bunschoten, M E Mingot-Castellano, N Ozdemir, I Pabinger, R Parra, J Pasi, K Peerlinck, A Rauch, V Roussel-Robert, M Serban, A Tagliaferri, J Windyga, E Zanon
BACKGROUND: Development of inhibitors is the most serious complication in haemophilia A treatment. The assessment of risk for inhibitor formation in new or modified factor concentrates is traditionally performed in previously treated patients (PTPs). However, evidence on risk factors for and natural history of inhibitors has been generated mostly in previously untreated patients (PUPs). The purpose of this study was to examine cases of de novo inhibitors in PTPs reported in the scientific literature and to the EUropean HAemophilia Safety Surveillance (EUHASS) programme, and explore determinants and course of inhibitor development...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28198996/treatment-burden-haemostatic-strategies-and-real-world-inhibitor-screening-practice-in-non-severe-haemophilia-a
#13
Paul Batty, Steve K Austin, Kate Khair, Carolyn M Millar, Ben Palmer, Savita Rangarajan, Jan-Phillip Stümpel, Murugaiyan Thanigaikumar, Thynn T Yee, Daniel P Hart
Inhibitor formation in non-severe haemophilia A is a life-long risk and associated with morbidity and mortality. There is a paucity of data to understand real-world inhibitor screening practice. We evaluated the treatment burden, haemostatic strategies, F8 genotyping and inhibitor screening practices in non-severe haemophilia A in seven London haemophilia centres. In the 2-year study period, 44% (377/853) patients received at least one haemostatic treatment. Seventy-nine percent of those treated (296/377) received factor VIII (FVIII) concentrate...
March 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28186089/adenovirus-mediated-tipe2-overexpression-inhibits-gastric-cancer-metastasis-via-reversal-of-epithelial-mesenchymal-transition
#14
H Yin, X Huang, M Tao, Q Hu, J Qiu, W Chen, J Wu, Y Xie
Tumor necrosis factor (TNF)-alpha-induced protein 8-like 2 (TNFAIP8L2; also termed TIPE2) has been shown to be involved in both the immune-negative modulation and cancer. We previously found that TIPE2 is lost in human gastric cancer, and TIPE2 restoration suppresses gastric cancer growth by induction of apoptosis and impairment of protein kinase B (PKB/AKT) and extracellular signal-regulated kinase-1/2 (ERK1/2) signaling. However, its correlation with epithelial-mesenchymal transition (EMT) in gastric cancer is largely elusive...
February 10, 2017: Cancer Gene Therapy
https://www.readbyqxmd.com/read/28166608/safety-efficacy-and-pharmacokinetics-of-rviii-singlechain-in-children-with-severe-hemophilia-a-results-of-a-multicenter-clinical-trial
#15
O Stasyshyn, C Djambas Khayat, G Iosava, J Ong, F Abdul Karim, K Fischer, A Veldman, N Blackman, K St Ledger, I Pabinger
Essentials rVIII-SingleChain is a novel recombinant factor VIII with covalently bonded heavy and light chains. Efficacy, safety and pharmacokinetics were studied in pediatric patients with severe hemophilia A. Across all prophylaxis regimens, the median annualized spontaneous bleeding rate was 0.00. rVIII-SingleChain showed excellent hemostatic efficacy and a favorable safety profile. SUMMARY: Background rVIII-SingleChain is a novel B-domain truncated recombinant factor VIII (rFVIII) comprised of covalently bonded FVIII heavy and light chains, demonstrating a high binding affinity to von Willebrand factor...
February 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28150393/acquired-hemophilia-a-manifesting-as-plasma-transfusion-uncontrolled-severe-bleeding-2-weeks-after-chorioamnionitis-induced-abortion
#16
Ayumi Matsuoka, Hiromasa Sasaki, Chiharu Sugimori, Shinya Hirabuki, Tsutomu Hoshiba, Hiroshi Fujiwara
Acquired hemophilia A (AHA) is a serious and rare complication of pregnancy, caused by autoantibodies to coagulation factor VIII after delivery. We here report the case of a 36-year-old primigravida woman who developed AHA following chorioamnionitis-caused miscarriage in the second trimester. Thirteen days after abortion, sudden, massive vaginal bleeding occurred with marked prolongation of activated partial thromboplastin time (APTT) in the absence of other abnormal coagulation data. Sequential transfusion of fresh frozen plasma did not achieve normalization of APTT...
February 2, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28150310/plasma-treated-with-amotosalen-and-ultraviolet-a-light-retains-activity-for-hemostasis-after-5-days-post-thaw-storage-at-1-to-6-o-c
#17
Anna Erickson, Katie Waldhaus, Tovo David, Norman Huang, Salvador Rico, Laurence Corash, Nina Mufti, Richard J Benjamin
BACKGROUND: Plasma thawed and stored at 1 to 6(°) C for up to 5 days (thawed plasma [TP]) provides rapid availability in emergencies and reduces plasma waste, but it carries risks of coagulation factor loss or activation, bacterial outgrowth, and viral contamination. We characterized changes in amotosalen/ultraviolet A (UVA) light pathogen-reduced, fresh-frozen plasma (FFP) and plasma frozen within 24 hours (PF24) with post-thaw storage. STUDY DESIGN AND METHODS: Amotosalen/UVA light-treated FFP and PF24 were thawed after approximately 3 to more than 12 months of frozen storage and held at 1 to 6(°) C for 5 days...
February 1, 2017: Transfusion
https://www.readbyqxmd.com/read/28135035/fibrinolysis-inhibitors-in-plaque-stability-a-morphological-association-pai-1-and-tafi-in-advanced-carotid-plaque
#18
A-C Jönsson Rylander, A Lindgren, J Deinum, G M L Bergström, G Böttcher, I Kalies, K Wåhlander
BACKGROUND: Fibrinolysis plays an important role in destabilization of atherosclerotic plaques and is tightly regulated by specific inhibitors. OBJECTIVE: The fibrinolysis inhibitors PAI-1 and TAFI were quantified and described in the morphological context of advanced carotid plaques (AHA VI-VIII) to elucidate their role in plaque stability. METHODS: Immunohistochemistry in serial sections along the longitudinal axis of endarterectomies from patients with symptomatic carotid stenosis (n=19) were studied using an antibody specific for free PAI-1 (I205), an antibody with high affinity for TAFI/TAFIa (CP17) and established antibodies for SMC (α-actin), endothelial cells (Von Willebrandt Factor, VWF), macrophages (CD68) and platelets (CD42)...
January 30, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28133956/development-of-a-novel-automated-screening-method-for-detection-of-fviii-inhibitors
#19
M S Evans, K J Donaldson, M E Eyster
INTRODUCTION: Factor VIII activity is routinely determined by measuring the activated partial thromboplastin time (aPTT) of a patient plasma sample and determining percent activity from a standard curve. To maximize the detection of a clotting factor inhibitor, a subjective assessment of parallelism of a patient curve compared with a standard curve is performed. We developed and validated an automated objective method to assess parallelism as a rapid screening tool for detection of an inhibitor to factor VIII during routine FVIII assays...
April 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28124406/recombinant-porcine-factor-viii-for-high-risk-surgery-in-paediatric-congenital-haemophilia-a-with-high-titre-inhibitor
#20
S E Croteau, Y L Abajas, A S Wolberg, B I Nielsen, G R Marx, C W Baird, E J Neufeld, P E Monahan
INTRODUCTION: High-titre factor VIII (FVIII) inhibitors complicate peri-operative haemostasis. Recombinant porcine FVIII (r-pFVIII) may provide an alternative haemostatic agent for high-risk procedures and allow FVIII activity monitoring. AIM: Devise an effective haemostatic plan for repair of a progressively symptomatic aortic coarctation in a 5-year-old male with immune tolerance induction (ITI) refractory high-titre FVIII inhibitors. METHODS: Preprocedure human FVIII inhibitor titre was 58 Bethesda Units mL(-1) (BU) and cross-reacted to neutralize porcine FVIII at 30 BU...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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