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https://www.readbyqxmd.com/read/29777571/optimization-of-pre-analytical-heat-treatment-for-inhibitor-detection-in-haemophilia-a
#1
P Batty, D P Hart, S Platton
INTRODUCTION: Factor VIII (FVIII) antibody formation is the greatest clinical and laboratory challenge within the haemophilia centre. The Nijmegen-Bethesda assay (NBA) is the gold standard for inhibitor quantification, but affected by pre-analytical variables including a patient's FVIII activity (FVIII:C). Pre-analytical heat treatment (PHT) provides a methodology for inhibitor testing when measurable FVIII:C is present. METHODS: We evaluated the effect of different PHT conditions (time/temperature) on FVIII:C as well as on potency of inhibitory activity in samples containing FVIII:C (endogenous pooled plasma and exogenous recombinant FVIII (rFL-FVIII) concentrate) or FVIII inhibitor...
May 18, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29776906/blood-induced-bone-loss-in-murine-hemophilic-arthropathy-is-prevented-by-blocking-the-irhom2-adam17-tnf%C3%AE-pathway
#2
Coline Haxaire, Narine Hakobyan, Tania Pannellini, Camila Carballo, David McIlwain, Tak W Mak, Scott Rodeo, Suchitra Acharya, Daniel Li, Jackie Szymonifka, Xiangqian Song, Sébastien Monette, Alok Srivastava, Jane E Salmon, Carl P Blobel
Hemophilic arthropathy (HA) is a debilitating degenerative joint disease that is a major manifestation of the bleeding disorder Hemophilia A. HA typically begins with hemophilic synovitis (HS) that resembles inflammatory arthritides such as rheumatoid arthritis (RA) and frequently results in bone loss in patients. A major cause of RA is inappropriate release of the pro-inflammatory cytokine tumor necrosis factor α (TNFα) by the TNFα convertase (TACE, also referred to as ADAM17) and its regulator, iRhom2...
May 18, 2018: Blood
https://www.readbyqxmd.com/read/29774166/hemostatic-state-augmented-with-platelet-indices-among-sudanese-diabetic-septic-foot
#3
Bashir Abdrhman Bashir, Mohamed Salih Ali
Background: Diabetes mellitus is a very rampant metabolic disorder, particularly type II. It has many complications such as the septic foot. Diabetic septic foot (DSF) patients are at high risk for coagulation abnormalities as well as surgical hazards. Owing to the potential sequelae of coagulation and vascular abnormalities, this work aimed at studying the hemostatic state and platelet indices in diabetes type II patients with septic foot. Methods: A case-control study was conducted during the period from July to September 2017 at Dr...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29769125/observational-study-of-coagulation-activation-in-early-breast-cancer-development-of-a-prognostic-model-based-on-data-from-the-real-world-setting
#4
Chiara Mandoj, Laura Pizzuti, Domenico Sergi, Isabella Sperduti, Marco Mazzotta, Luigi Di Lauro, Antonella Amodio, Silvia Carpano, Anna Di Benedetto, Claudio Botti, Francesca Ferranti, Anna Antenucci, Maria Gabriella D'Alessandro, Paolo Marchetti, Silverio Tomao, Giuseppe Sanguineti, Antonio Giordano, Marcello Maugeri-Saccà, Gennaro Ciliberto, Laura Conti, Patrizia Vici, Maddalena Barba
BACKGROUND: Cancer and coagulation activation are tightly related. The extent to which factors related to both these pathologic conditions concur to patient prognosis intensely animates the inherent research areas. The study herein presented aimed to the development of a tool for the assessment and stratification of risk of death and disease recurrence in early breast cancer. METHODS: Between 2008 and 2010, two hundreds thirty-five (N: 235) patients diagnosed with stage I-IIA breast cancer were included...
May 16, 2018: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29767844/comorbidities-associated-with-higher-von-willebrand-factor-vwf-levels-may-explain-the-age-related-increase-of-vwf-in-von-willebrand-disease
#5
Ferdows Atiq, Karina Meijer, Jeroen Eikenboom, Karin Fijnvandraat, Eveline P Mauser-Bunschoten, Karin P M van Galen, Marten R Nijziel, Paula F Ypma, Joke de Meris, Britta A P Laros-van Gorkom, Johanna G van der Bom, Moniek P de Maat, Marjon H Cnossen, Frank W G Leebeek
Some comorbidities, such as hypertension, are associated with higher von Willebrand factor (VWF) levels in the general population. No studies have been conducted to assess this association in patients with von Willebrand disease (VWD). Therefore, we studied this association in patients with type 1 (n = 333) and type 2 (n = 203) VWD from the 'WiN" study. VWF antigen (VWF:Ag) was higher in type 1 VWD patients with hypertension [difference: 0·23 iu/ml, 95% confidence interval (CI): 0·11-0·35], diabetes mellitus (0·11 iu/ml, 95% CI: -0·02 to 0·23), cancer (0·14 iu/ml, 95% CI: 0·03-0·25) and thyroid dysfunction (0·14 iu/ml, 95% CI: 0·03-0·26) than in patients without these comorbidities (all corrected for age, sex and blood group)...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29754468/bleeding-score-in-type-1-von-willebrand-disease-patients-using-the-condensed-mcmdm-1-vwd-validated-questionnaire
#6
A Pathare, F Al Hajri, S Al Omrani, N Al Obaidani, B Al Balushi, K Al Falahi
BACKGROUND: Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. AIMS: To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. METHODS: Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator...
May 13, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29751951/biochemical-characterization-and-immunogenicity-of-neureight-a-recombinant-full-length-factor-viii-produced-by-fed-batch-process-in-disposable-bioreactors
#7
Sandrine Delignat, Ivan Peyron, Maria El Ghazaly, Srinivas V Kaveri, Jan Rohde, Frank Mueller, Sebastien Lacroix-Desmazes
Hemophilia A is a X-linked recessive bleeding disorder consecutive to the lack of circulating pro-coagulant factor VIII (FVIII). The most efficient strategy to treat or prevent bleeding in patients with hemophilia A relies on replacement therapy using exogenous FVIII. Commercially available recombinant FVIII are produced using an expensive perfusion technology in stainless steel fermenters. A fed-batch fermentation technology was recently developed to produce 'Neureight', a full-length recombinant human FVIII, in Chinese hamster ovary (CHO) cells...
May 5, 2018: Cellular Immunology
https://www.readbyqxmd.com/read/29749544/repair-of-urethral-defects-by-an-adipose-mesenchymal-stem-cell%C3%A2-porous-silk-fibroin-material
#8
Binqiang Tian, Lujie Song, Tao Liang, Zuowei Li, Xuxiao Ye, Qiang Fu, Yonghui Li
The aim of the present study was to determine whether it was possible to repair urethral defects with a material of adipose mesenchymal stem cells (ADMSCs)‑porous silk fibroin (SF). A total of 39 male New Zealand white rabbits were randomly divided into a control group, an SF group and a bromodeoxyuridine (BrdU)‑labeled ADMSCs‑SF group (SSF group; n=13/group). Defects were made by resecting the posterior urethral wall. The defects in the SF and SSF groups were repaired using SF and BrdU‑labeled ADMSCs‑SF materials respectively...
May 9, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29747114/effect-of-double-filtration-plasmapheresis-for-antibody-mediated-rejection-on-hemostasis-parameters-and-thrombin-generation
#9
R Marlu, P Malvezzi, L Seyve, T Jouve, J Maurizi, F Defendi, P L Carron, M Christophe, A Le Gouellec, B Polack, L Rostaing
INTRODUCTION: Donor-specific alloantibodies (DSAs) cause kidney-allograft loss in chronic antibody-mediated rejection (CAMR). Treatment relies on blocking antibody-producing cells and removing DSAs by apheresis: e.g., double-filtration plasmapheresis (DFPP). MATERIALS AND METHODS: To determine the impact of DFPP (6 or 8 sessions/patient) on clotting factors and natural anticoagulants, and on thrombin generation, we performed a prospective and observational study in five CAMR kidney-transplant patients who received DFPP plus rituximab therapy...
April 20, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29743396/-congenital-factor-v-and-factor-viii-deficiency-discovered-in-an-elderly-patient-with-abnormal-bleeding-after-trauma
#10
Yoshiyuki Ogawa, Kunio Yanagisawa, Yuri Uchiyama, Akira Matsumoto, Madoka Inoue, Kohtaro Toyama, Yuri Miyazawa, Naomichi Matsumoto, Hiroshi Handa
Congenital combined deficiency of coagulation factor V (FV) and factor VIII (FVIII) (F5F8D) is a rare autosomal recessive bleeding disorder caused by mutations in lectin mannose-binding type 1 (LMAN1) or multiple coagulation factor deficiency 2 (MCFD2) encoding chaperone molecules involved in the intracellular transport of FV and FVIII. Here, we report a case of F5F8D in an elderly patient diagnosed with hematoma after a right thigh injury. A 71-year-old male had a history of abnormal bleeding after tooth extraction and cholecystectomy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29742318/a-comparative-analysis-of-different-automated-von-willebrand-factor-glycoprotein-ib-binding-activity-assays-in-well-typed-von-willebrand-disease-patients
#11
I Vangenechten, K Mayger, P Smejkal, O Zapletal, J J Michiels, G W Moore, A Gadisseur
BACKGROUND: von Willebrand Disease (VWD) is an inherited bleeding disorder caused by quantitative (type 1, 3) or qualitative (type 2) von Willebrand factor (VWF) defect. VWD diagnosis and classification require numerous laboratory tests. VWF: Glycoprotein Ib (GPIb)-binding activity assays are used to distinguish type 1 from type 2 VWD. OBJECTIVES: Three different automated VWF:GPIb-binding activity assays were compared. PATIENTS AND METHODS: BC-VWF:RCo (Siemens Healthcare Diagnostics), HemosIL® VWF:RCo (Instrumentation Laboratory) and INNOVANCE® VWF:Ac (Siemens Healthcare Diagnostics) were performed in a well typed VWD cohort (n=142)...
May 9, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29739073/a-case-of-acquired-hemophilia-a-usefulness-of-various-methods-for-judging-mixing-test-results-for-monitoring-the-effect-of-immunosuppressive-therapy
#12
Ryosuke Moriai, Nozomi Yanagihara, Akemi Endoh, Satoru Yamada, Maki Mochizuki, Takashi Kondo, Teruo Endoh, Koichi Asanuma, Satoshi Takahashi
BACKGROUND: Measurement of FVIII inhibitor (FVIII INH) levels is important for determining the effect of immunosuppressive therapy on acquired hemophilia A (AHA). However, FVIII INH can only be measured at a limited number of laboratories, which means that there are delays in obtaining the results at many sites. METHODS: A series of mixing tests were carried out in a case of AHA, followed by comparison of various methods for judging the obtained results in association with a change of FVIII INH...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737459/safety-of-intra-articular-transplantation-of-lentivirally-transduced-mesenchymal-stromal-cells-for-haemophilic-arthropathy-in-a-non-human-primate
#13
Tsukasa Ohmori, Hiroaki Mizukami, Yuko Katakai, Sho Kawai, Hitoyasu Nakamura, Makoto Inoue, Tsugumine Shu, Hideharu Sugimoto, Yoichi Sakata
Joint bleeding and resultant arthropathy are major determinants of quality of life in haemophilia patients. We previously developed a mesenchymal stromal cell (MSC)-based treatment approach for haemophilic arthropathy in a mouse model of haemophilia A. Here, we evaluated the long-term safety of intra-articular injection of lentivirally transduced autologous MSCs in non-human primates. Autologous bone-marrow-derived MSCs transduced with a lentiviral vector expressing coagulation factor VIII (FVIII) were injected into the left knee joint of cynomolgus monkeys...
May 8, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29737426/elevated-factor-viii-increases-the-risk-of-cerebral-venous-thrombosis-a-case-control-study
#14
Loes Vecht, Susanna M Zuurbier, Joost C M Meijers, Jonathan M Coutinho
BACKGROUND: Elevated factor VIII (FVIII) is a risk factor for leg-vein thrombosis and pulmonary embolism. We assessed whether elevated FVIII is also a risk factor for cerebral venous thrombosis (CVT). METHODS: We performed a matched case-control study. We assessed patients with CVT, as cases, admitted between July 2006 and December 2016. The controls were healthy hospital-staff employees matched for age (within 5 years) and sex. FVIII activity was measured at least 3 months after CVT diagnosis...
May 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29735583/analysis-of-mcfd2-and-lman1-deficient-mice-demonstrates-distinct-functions-in-vivo
#15
Min Zhu, Chunlei Zheng, Wei Wei, Lesley Everett, David Ginsburg, Bin Zhang
The LMAN1-MCFD2 complex serves as a cargo receptor for efficient transport of factor V (FV) and FVIII from the endoplasmic reticulum (ER) to the Golgi. Genetic deficiency of LMAN1 or MCFD2 in humans results in the moderate bleeding disorder combined FV and FVIII deficiency, with a similar phenotype previously observed in LMAN1-deficient mice. We now report that MCFD2-deficient mice generated by gene targeting also demonstrate reduced plasma FV and FVIII, with levels lower than those in LMAN1-deficient mice, similar to previous observations in LMAN1- and MCDF2-deficient humans...
May 8, 2018: Blood Advances
https://www.readbyqxmd.com/read/29735454/-prophylactic-treatment-with-low-and-intermediate-dose-factor-viii-in-children-with-severe-hemophilia-a-comprehensive-evaluation-of-joint-outcomes-and-correlation-analysis
#16
Jin-Mu Zhuang, Xue-Yan Sun, Xuan Zhou, Zhu-Qin Liu, Jing Sun
OBJECTIVE: To study the effect of low- and intermediate-dose factor VIII (FVIII) for prophylactic treatment of severe hemophilia A in children by comprehensively evaluating the outcomes of the joints. METHODS: Forty-seven children with severe hemophilia A (FVIII activity ≤2%) were enrolled in this study. Eighteen of the children received prophylactic treatment with low-dose FVIII (10 U/kg, 2-3 times a week), 20 received prophylactic treatment with intermediate-dose FVIII (15-30 U/kg, 3 times a week), and 9 received on-demand treatment with FVIII infusion when bleeding occurred according to the Chinese Expert Consensus on the Diagnosis and Treatment of Hemophilia...
April 20, 2018: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/29734520/routine-measurements-of-factor-viii-activity-and-inhibitor-titer-in-the-presence-of-emicizumab-utilizing-anti-idiotype-monoclonal-antibodies
#17
Keiji Nogami, Tetsuhiro Soeda, Tomoko Matsumoto, Yoshiki Kawabe, Takehisa Kitazawa, Midori Shima
BACKGROUND: Emicizumab is an anti-factor (F)IXa/X bispecific monoclonal antibody (mAb), mimicking the Factor (F)VIIIa cofactor activity. Emicizumab does not require activation by thrombin and its shortening effect on the activated partial prothrombin time (aPTT) is more pronounced than that of Factor (F)VIII. aPTT-based FVIII activity (FVIII:C) and FVIII inhibiter titer measurements are influenced by the presence of emicizumab. AIM: To establish a reliable aPTT-based assay to measure FVIII in the presence of emicizumab...
May 7, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29732985/treatment-of-chronic-articular-pain-in-adults-with-hemophilia
#18
E Carlos Rodriguez-Merchan
INTRODUCTION: Adult people with hemophilia (PWH) mainly use Factor VIII/Factor IX (FVIII/FIX) to lessen chronic articular pain, followed in frequency by nonsteroidal anti-inflammatory drugs. Analgesics are used by only one-third of adult PWH. Limitations in activities of daily living are encountered in the large majority of PWH, and most describe pain as affecting their state of mind. A review of the literature on their treatment is important because chronic pain in adult PWH is often undertreated or wrongly treated, causing psychological problems for these patients...
May 3, 2018: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/29732708/pharmacokinetic-modelling-and-validation-of-the-half-life-extension-needed-to-reduce-the-burden-of-infusions-compared-with-standard-factor-viii
#19
C Hermans, J Mahlangu, J Booth, H Schütz, E Santagostino, G Young, H-Y Lee, K N Steinitz-Trost, V Blanchette, E Berntorp
INTRODUCTION: Currently, no universally accepted definition of extended half-life (EHL) recombinant FVIII (rFVIII) exists. Identifying the minimum half-life extension ratio required for a reduction in dosing frequency compared with standard rFVIII could enable a more practical approach to decisions around prophylaxis with EHL rFVIII. AIM: To identify the half-life extension ratio required to decrease rFVIII dosing frequency by at least 1 day while maintaining the proportion of patients with plasma rFVIII levels above 1 IU/dL and without increasing the total weekly dose...
May 6, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29727892/hemostasis-during-extreme-exertion
#20
Cécile Hélène Kicken, Adam Miszta, Hilde Kelchtermans, Bas De Laat
Exercise is protective against cardiovascular disease, but can also provoke sudden cardiac death, a phenomenon referred to as "the exercise paradox." Extreme exertion is known to induce a rebalanced hemostatic state by causing hypercoagulability and concomitantly enhanced fibrinolysis. Over the past decade, novel techniques for quantifying hemostasis have been introduced, which may provide new insights into this process. This review summarizes recent literature on the effect of extreme exertion of both short and long duration on coagulation, fibrinolysis, and recovery of hemostatic balance...
May 4, 2018: Seminars in Thrombosis and Hemostasis
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