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Factor VIII inhibitor

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https://www.readbyqxmd.com/read/28428025/adipokines-as-atherothrombotic-risk-factors-in-obese-subjects-associations-with-haemostatic-markers-and-common-carotid-wall%C3%A2-thickness
#1
É Csongrádi, M Káplár, B Nagy, C A Koch, A Juhász, L Bajnok, Z Varga, I Seres, Z Karányi, M T Magyar, L Oláh, A Facskó, J Kappelmayer, G Paragh
BACKGROUND AND AIMS: Some crucial associations between obesity-related altered adipokine levels and the main factors of atherosclerotic, atherothrombotic processes are not fully known. We analysed the relationships of classic adipokines, namely leptin, resistin, adiponectin, tumour necrosis factor-alpha (TNF-α), interleukin 6 (IL-6) with the markers of platelet activation, including mean platelet volume (MPV), platelet surface/soluble P-selectin, platelet-derived microparticles (PMPs), the parameters of coagulation abnormalities and common carotid intima-media thickness (IMT) in obese patients with or without atherosclerotic comorbidities in comparison to age- and sex-matched controls...
March 8, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/28420167/treatment-and-prevention-of-bleeds-in-haemophilia-patients-with-inhibitors-to-factor-viii-ix
#2
REVIEW
Angiola Rocino, Massimo Franchini, Antonio Coppola
The development of alloantibodies neutralising therapeutically administered factor (F) VIII/IX (inhibitors) is currently the most severe complication of the treatment of haemophilia. When persistent and at a high titre, inhibitors preclude the standard replacement treatment with FVIII/FIX concentrates, making patients' management challenging. Indeed, the efficacy of bypassing agents, i.e., activated prothrombin complex concentrates (aPCC) and recombinant activated factor VII (rFVIIa), needed to overcome the haemostatic interference of the inhibitor, is not comparable to that of factor concentrates...
April 17, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28401658/kreuth-iv-european-consensus-proposals-for-treatment-of-haemophilia-with-coagulation-factor-concentrates
#3
P L F Giangrande, F Peyvandi, B O'Mahony, M-E Behr-Gross, A Hilger, W Schramm, P M Mannucci
INTRODUCTION: This report summarizes recommendations relating to haemophilia therapy arising from discussions among experts from 36 European countries during the 'Kreuth IV' meeting in May 2016. AIM: The objective of the meeting was for experts in the field of haemophilia from across Europe to draft resolutions regarding current issues relating to the treatment of haemophilia. RESULTS: Hospitals providing clinical care for people with haemophilia and related disorders are strongly recommended to seek formal designation as either European Haemophilia Treatment Centres (EHTC) or European Haemophilia Comprehensive Care Centres (EHCCC)...
April 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28393618/regulatory-b-cells-are-functionally-impaired-in-patients-having-hemophilia-a-with-inhibitors
#4
Mohamed-Rachid Boulassel, Maryam Al-Ghonimi, Badriya Al-Balushi, Amal Al-Naamani, Zahra Al-Qarni, Yasser Wali, Mohamed Elshinawy, Maryam Al-Shezawi, Hamad Khan, Hanan Nazir, Doaa Khater, Anil Pathare, Salam Al-Kindi
Development of inhibitors remains a major clinical complication in patients with hemophilia A receiving replacement therapy with factor VIII (FVIII). Understanding the immune mechanisms involved in the development of inhibitors can provide valuable information about pathways to human tolerance. Recent evidence indicates that B regulatory (Breg) cells play a pivotal role in controlling the production of antibodies (Abs) while promoting follicular T helper (Tfh) cells and monocytes, expressing the low-density lipoprotein receptor-related protein (LRP/CD91), which is involved in FVIII intake from the circulation...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28393468/the-association-between-health-utility-and-joint-status-among-people-with-severe-haemophilia-a-findings-from-the-kappa-register
#5
M Osooli, K Steen Carlsson, F Baghaei, M Holmström, S Rauchensteiner, P A Holme, L Hvitfeldt, J Astermark, E Berntorp
INTRODUCTION: People with severe haemophilia A have reportedly impaired health related quality of life (utility) mainly due to recurrent bleeding, arthropathy and treatment burden. AIM: To estimate utilities and evaluate their potential correlates - most importantly the joint status - among people with severe haemophilia A. METHODS: In this cross-sectional study, eligible participants had severe haemophilia A, were aged ≥15, negative for factor VIII inhibitor and included in the KAPPA register of Denmark, Norway and Sweden...
April 10, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28387451/large-scale-studies-assessing-anti-factor-viii-antibody-development-in-previously-untreated-haemophilia-a-what-has-been-learned-what-to-believe-and-how-to-learn-more
#6
REVIEW
Alfonso Iorio, Kathelijn Fischer, Michael Makris
Minimizing the risk of inhibitor development by acting on modifiable risk factors remains a sensible goal for treatment optimization in haemophilia A. By critically appraising published studies assessing inhibitor development, this review addresses the role of studies in previously untreated patients (PUPs) for establishing the immunogenicity of new concentrates, suggest novel research design to be adopted in future studies and discuss clinical practice implications of the reported differential immunogenicity of Kogenate Bayer and Advate factor VIII concentrates...
April 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28381691/autoimmune-bullous-disease-and-hashimoto-s-disease-complicated-by-acquired-hemophilia-a
#7
Nobuko Nishiura, Daisuke Ujimoto, Jiro Fujita, Tetsuo Maeda, Yukinobu Nakagawa, Hirokazu Kashiwagi, Kenji Oritani, Yoshiaki Tomiyama, Yuzuru Kanakura
A 67-year-old man was admitted with a 1-month history of spontaneous hematoma in his right back and severe anemia. He had suffered from rashes with blisters involving both legs for 10 years, which had shown worsening and extended to his entire body concurrently with the hematoma. APTT was markedly prolonged to 119 seconds, and Factor VIII:C and FVIII inhibitor levels were less than 1% and 153.1 BU/ml, respectively, confirming the diagnosis of acquired hemophilia A (AHA). Skin biopsy revealed his rashes to be caused by autoimmune bullous disease (ABD), and laboratory and physical findings showed that he also had autoimmune hypothyroidism (Hashimoto's disease)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28365649/clinical-application-of-prothrombin-complex-concentrate-in-blood-management-in-patients
#8
Sherri Ozawa, Tiffany Nelson
Management of patients receiving anticoagulants is a major factor in achieving better outcomes. Anticoagulant therapy may need to be discontinued or rapidly reversed before urgent surgery or invasive procedures. In these situations, treatment with concentrated vitamin K, fresh frozen plasma, and/or clotting factors can achieve more rapid anticoagulant reversal than can drug discontinuation alone. Activated prothrombin complex concentrate is used to treat hemophiliac patients with acquired factor VIII inhibitors...
April 2017: Critical Care Nurse
https://www.readbyqxmd.com/read/28352491/acquired-factor-viii-deficiency-two-case-reports-and-a-review-of-literature
#9
Lan Mo, George C Bao
BACKGROUND: Acquired factor VIII (FVIII) deficiency, or acquired hemophilia A (AHA), is a rare autoimmune disorder involving antibody-mediated depletion of coagulation FVIII, leading to severe, life-threatening bleeding. The condition is often associated with other autoimmune disorders, and its treatment involves replacement of FVIII and various modes of immunosuppression. Recently, a few noteworthy therapeutic advances have been made. We present two cases of severe AHA in Chinese women...
2017: Experimental Hematology & Oncology
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
#10
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
#11
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
#12
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 C Gerstenfeld, Dana T 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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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)
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