keyword
MENU ▼
Read by QxMD icon Read
search

FVIII

keyword
https://www.readbyqxmd.com/read/28641667/-research-progress-on-genetic-factors-and-hemophilia-a-clotting-factor-inhibitor-review
#1
Zheng-Bin Hu, Xin Sun, Li-Ya He
Hemophilia A (Hemophilia A, HA) is an X-linked recessive hereditary coagulation function disorder, the deficiency and dysfunction of blood coagulation were caused by the mutations of gene encoding clotting factor VIII. The treatment of hemophilia A still depends on the replacement therapy with blood coagulation factor. However, the repeated infusion of clotting factor will produce the neutralizing antibody against FVIII, then resulting in one of the serious complications. The reports on the incidence of inhibitor are different at home and abroad...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641362/estimating-the-potential-cost-of-a-high-dose-immune-tolerance-induction-iti-therapy-relative-to-the-cost-of-a-combined-therapy-of-a-low-dose-iti-therapy-with-bypassing-agent-prophylaxis
#2
G Kenet, A Oladapo, J D Epstein, C Thompson, A Novack, D J Nugent
INTRODUCTION: The International Immune Tolerance Study (I-ITI) demonstrated comparable success rates between low (FVIII 50 IU/kg/TIW) and high dose (FVIII 200 IU/kg/day) regimens. While costlier, the high dose ITI regimen achieved shorter time-to-treatment success with fewer bleeding episodes compared to the low dose ITI regimen. Adding bypassing agent prophylaxis (BAP) to a low dose ITI regimen may reduce bleeding while still being less costly than high dose ITI. AIM AND METHODS: An economic model was developed to compare high dose ITI to low dose ITI with BAP...
June 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28636264/desmopressin-in-haemophilia-the-need-for-a-standardised-clinical-response-and-individualised-test-regimen
#3
S C M Stoof, L M Schütte, F W G Leebeek, M H Cnossen, M J H A Kruip
INTRODUCTION: Due to interindividual variation in desmopressin response, non-severe haemophilia A patients require desmopressin testing prior to therapeutic treatment. However, adequate response or frequency of blood sampling is not standardised in international guidelines. Consequently, various definitions and blood sampling protocols are currently applied. Interestingly, sustainability of desmopressin response is not incorporated into these definitions. AIM: To study desmopressin response rates in a cohort of non-severe haemophilia A patients using currently accepted desmopressin response definitions...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28628153/-13-cases-of-littoral-cell-angioma-in-spleens
#4
(no author information available yet)
OBJECTIVE: To investigate the clinicopathological features, morphological characteristics, immunophenotypes of littoral cell angioma (LCA) in spleen, and to provide new evidence for making diagnosis and avoiding misdiagnosis. METHODS: Clinicopathological data, histological characteristics of 13 cases of LCA were retrospectively studied and immunohistochemical staining was imposed on the paraffi-nembedded specimens, and 5 cases of cavernous hemangioma, 4 cases of normal littoral cells of spleens were used as control groups, simultaneously...
June 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28612430/igg-subclasses-of-fviii-inhibitors-in-an-argentine-cohort-of-severe-hemophilia-a-patients-analysis-by-flow-cytometry
#5
M B Irigoyen, L Primiani, M Felippo, M Candela, M M E de Bracco, N V Galassi
INTRODUCTION: FVIII inhibitors consist of a polyclonal population of antibodies. Previous studies have demonstrated different distribution of IgG subclasses. IgG4 was associated to high level of FVIII inhibitors and failure of immune tolerance induction (ITI) treatment. This study monitored the relative distribution of IgG subclasses of anti-FVIII in patients with severe hemophilia A (SHA). METHODS: Anti-FVIII antibodies were measured employing an immunomethod, developed in our laboratory, that combines flow cytometry (FC) with microspheres coupled (FVIII-m) or not (Control-m) to FVIII...
June 14, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28609796/successful-use-of-a-highly-purified-plasma-von-willebrand-factor-concentrate-containing-little-fviii-for-the-long-term-prophylaxis-of-severe-type-3-von-willebrand-s-disease
#6
Tomas Simurda, Miroslava Dobrotova, Ingrid Skornova, Juraj Sokol, Peter Kubisz, Jan Stasko
No abstract text is available yet for this article.
June 13, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28602126/clinical-and-prognostic-significance-of-lupus-anticoagulant-measurement-in-patients-with-lung-cancer
#7
Xianming Fei, Huan Wang, Lei Jiang, Tongwei Zhao, Maoliang Cheng, Wufeng Yuan
Lupus anticoagulants is related to both recurrent thrombosis and cancer. Thrombotic complications occur more frequently in patients with lung cancer. The aim of this study is to investigate the association of lupus anticoagulants with hypercoagulability and thrombotic complications, as well as prognostic significance of lupus anticoagulants for patients with lung cancer. The study comprised 205 patients with non-small cell lung cancer. Plasma normalized LAC ratio, D-dimer, fibrinogen, activities of antithrombin, and FVIII before treatment were analyzed by coagulation analyzer, and routine hematologic and biochemical parameters were also evaluated...
January 1, 2017: Technology in Cancer Research & Treatment
https://www.readbyqxmd.com/read/28601434/an-open-label-single-dose-pharmacokinetic-study-of-factor-viii-activity-after-administration-of-moroctocog-alfa-af-cc-in-male-chinese-patients-with-hemophilia-a
#8
Hongzhong Liu, Runhui Wu, Pei Hu, Feifei Sun, Lihong Xu, Yali Liang, Sunil Nepal, Peng Roger Qu, Francois Huard, Joan M Korth-Bradley
PURPOSE: Hemophilia A represents up to 80% of all hemophilia cases in China. In patients with this condition, bleeding can be prevented and controlled by administering clotting factor VIII (FVIII). Since their initial availability, recombinant FVIII products have undergone several iterations to enhance their safety. Moroctocog alfa albumin-free cell culture (AF-CC) is among the third generation of recombinant FVIII products and received regulatory approval in China in August 2012. The present study characterizes the single-dose pharmacokinetic parameters of FVIII activity (FVIII:C) after administration of moroctocog alfa (AF-CC) in male Chinese patients with hemophilia A...
June 7, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28599169/modifiers-of-clinical-phenotype-in-severe-congenital-hemophilia
#9
Massimo Franchini, Pier Mannuccio Mannucci
Patients with inherited hemophilia A and B usually exhibit a bleeding tendency of a severity proportional to the degree of plasmatic deficiency of the coagulant activity of factor VIII (FVIII:C) and factor IX (FIX:C). Although patients with severe hemophilia (i.e., with FVIII:C and FIX:C levels <1IU/dL) are generally those with the most severe bleeding phenotype, it is common experience that a variable proportion of them experiences a milder bleeding tendency. In this review, we summarize the current knowledge on the possible mechanisms at the basis of the phenotypic heterogeneity of severe hemophilia, focusing in particular on the role of FVIII/FIX gene mutations and thrombophilic polymorphisms...
June 1, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28597369/first-report-of-real-time-monitoring-of-coagulation-function-potential-and-igg-subtype-of-anti-fviii-autoantibodies-in-a-child-with-acquired-hemophilia-a-associated-with-streptococcal-infection-and-amoxicillin
#10
Masahiro Takeyama, Keiji Nogami, Takahiro Kajimoto, Kenichi Ogiwara, Tomoko Matsumoto, Midori Shima
We describe an 8-year-old boy with acquired hemophilia A (AHA) associated with streptococcal infection and amoxicillin. Laboratory data revealed low factor VIII activity (FVIII:C, 1.5 IU/dl), and FVIII inhibitor (15.9 BU/ml). Comprehensive coagulation function assays, including rotation thromboelastometry (ROTEM(®)), revealed a markedly prolonged clotting time. Thrombin and plasmin generation (TG/PG) appeared to be moderately impaired. The inhibitor epitope of his anti-FVIII autoantibody recognized light and heavy chains...
June 8, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28596657/increased-level-of-factor-viii-and-physiological-inhibitors-of-coagulation-in-patients-with-sickle-cell-disease
#11
Mohamed Chekkal, Mohamed Chakib Arslane Rahal, Khedidja Moulasserdoun, Fatima Seghier
Sickle cell disease (SCD) is a hemoglobinopathy characterized by hemolysis, oxidative stress, and vaso-occlusive crises. Thromboembolism also remains a serious complication and probably underestimated in the SCD. Our objective was to seek the existence of hemostasis abnormalities that predispose to thrombosis such as elevation of FVIII and Physiological inhibitors of coagulation deficiency. We studied 81 patients with SCD, including 32 homozygous S/S, 20 double heterozygous S/β thalassemia and 29 heterozygous S/A...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28581694/abnormal-von-willebrand-factor-secretion-factor-viii-stabilization-and-thrombus-dynamics-in-type-2n-von-willebrand-disease-mice
#12
Laura L Swystun, Ilinca Georgescu, Jeff Mewburn, Meghan Deforest, Kate Nesbitt, Kassandra Hebert, Courtney Dwyer, Christine Brown, Colleen Notley, David Lillicrap
BACKGROUND: von Willebrand factor (VWF) and factor VIII (FVIII) circulate as a non-covalent complex with VWF serving as the carrier for FVIII. VWF indirectly influences secondary hemostasis by stabilizing FVIII and transporting it to the site of primary hemostasis. Type 2N von Willebrand disease involves impaired binding of VWF to FVIII, resulting in decreased plasma levels of FVIII. OBJECTIVES: In these studies, we characterize the impact of three type 2N VWD variants (R763A, R854Q, R816W) on VWF secretion, FVIII stabilization, and thrombus formation in a murine model...
June 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28576889/blood-coagulation-system-in-patients-with-chronic-kidney-disease-a-prospective-observational-study
#13
Meng-Jie Huang, Ri-Bao Wei, Yang Wang, Ting-Yu Su, Ping Di, Qing-Ping Li, Xi Yang, Ping Li, Xiang-Mei Chen
OBJECTIVES: Thromboembolic events are the major factor affecting the prognosis of patients with chronic kidney disease (CKD). Haemostatic alterations are possible causes of these complications, but their roles remain poorly characterised. In the prospective observational study, we investigated the entire coagulation process in patients with CKD to elucidate the mechanisms of their high thromboembolic risk. METHODS: A total of 95 patients with CKD and 20 healthy controls who met the inclusion criteria were consecutively recruited from September 2015 to March 2016...
June 1, 2017: BMJ Open
https://www.readbyqxmd.com/read/28574805/the-development-of-fviii-inhibitors-in-relation-to-il10-gene-polymorphism-in-hemophilia-a-egyptian-pediatric-patients
#14
Hoda Sadek, Ilham Youssry, Nihal Salah Eldeen Ibrahim, Amany Ahmed Abou-Elalla, Gehad Atef, Somaia Mohammed Mousa
BACKGROUND: Development of inhibitors against Factor VIII (FVIII) in hemophilia A patients is a serious complication of therapy. Many cytokines, including interleukin-10 (IL10), may affect inhibitor development; however, literature data are not sufficient to prove this association. The aim of this study was to investigate the relation between FVIII inhibitor formation and IL10-1082A/G polymorphism among Egyptian hemophiliacs. METHODS: Patients were screened for FVIII inhibitors using the Bethesda method...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#15
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
June 1, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28561280/anti-fc%C3%AE-riib-cd32-antibodies-differentially-modulate-murine-fviii-specific-recall-response-in-vitro
#16
Nadine Vollack, Julia Friese, Sabine Bergmann, Mark S Cragg, Andreas Tiede, Sonja Werwitzke
Fc gamma receptors (FcγRs) for IgG regulate adaptive immune responses by modulating activating and inhibitory signaling pathways within immune cells. Data from a hemophilia A mouse model demonstrate that genetic deletion or blockade of the inhibitory FcγR (CD32) suppresses the formation of antibody secreting cells (ASCs) in vitro. Mechanisms preventing the FVIII-specific recall response, however, remain unclear. Here, the potential role of CD32 inhibition was studied by differentially modulating receptor activity with selected anti-CD32 monoclonal antibodies (mAbs)...
May 31, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28558995/cellular-uptake-of-coagulation-factor-viii-elusive-role-of-the-membrane-binding-spikes-in-the-c1-domain
#17
Lydia Castro-Núñez, Johanna M Koornneef, Mariska G Rondaij, Esther Bloem, Carmen van der Zwaan, Koen Mertens, Alexander B Meijer, Henriet Meems
Low density lipoprotein receptor-related protein 1 (LRP1) is involved in the catabolism of many ligands, including factor VIII (FVIII) and alpha-2-macroglobulin (α2M). Transfer of FVIII to LRP1 is currently believed to be preceded by pre-concentration on the cell surface, by interacting with a so far unidentified component. In the present study, we used confocal microscopy and flow cytometry to compare endocytosis of FVIII and α2M using U87MG cells. The results show that α2M is rapidly internalized and does not compete for LRP1 mediated internalization of FVIII...
May 27, 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/28552407/a-novel-platform-for-immune-tolerance-induction-in-hemophilia-a-mice
#18
Simone Merlin, Elvira Stefania Cannizzo, Ester Borroni, Valentina Bruscaggin, Piercarla Schinco, Warut Tulalamba, Marinee K Chuah, Valder R Arruda, Thierry VandenDriessche, Maria Prat, Guido Valente, Antonia Follenzi
Hemophilia A (HA) is an X-linked bleeding disease caused by factor VIII (FVIII) deficiency. We previously demonstrated that FVIII is produced specifically in liver sinusoid endothelial cells (LSECs) and to some degree in myeloid cells, and thus, in the present work, we seek to restrict the expression of FVIII transgene to these cells using cell-specific promoters. With this approach, we aim to limit immune response in a mouse model by lentiviral vector (LV)-mediated gene therapy encoding FVIII. To increase the target specificity of FVIII expression, we included miRNA target sequences (miRTs) (i...
May 26, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28551548/quantitative-evaluation-of-atherosclerotic-plaques-and-intraplaque-neovascularization-using-contrast-enhanced-ultrasound-after-treatment-with-atorvastatin-in-rabbits
#19
Hong-Yu Liu, Jia Zhou, Hai Tong, Yan Tang, Xiao-Fang Wang, Qi-Chang Zhou
INTRODUCTION: This study is supposed to investigate the value of contrast-enhanced ultrasound (CEUS) in quantitative evaluation of atherosclerotic plaques and intraplaque neovascularization after treatment with atorvastatin (ATV) in rabbits. MATERIAL AND METHODS: Forty-five New Zealand white rabbits were enrolled to construct the rabbit model of AS. All rabbits were assigned into the control, AS group and ATV groups (n=15 individually). The AS plaque formation and relative parameters were observed and calculated by CEUS respectively...
May 25, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28550758/platelets-and-hemophilia-a-review-of-the-literature
#20
REVIEW
Julia Riedl, Cihan Ay, Ingrid Pabinger
Hemophilia A and B are inherited bleeding disorders due to deficiencies of the clotting factors VIII and IX, respectively. The severity of the disease correlates with remaining factor levels, although individual differences in bleeding tendency are seen despite similar factor levels. While thrombin generation is severely impaired in persons with hemophilia, primary hemostasis, i.e. platelet function, has been generally considered to be normal. However, some studies reported prolonged bleeding times in hemophilia, suggesting that also primary hemostasis is affected...
July 2017: Thrombosis Research
keyword
keyword
45427
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"