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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/29775658/hydroxysafflor-yellow-a-protects-brain-microvascular-endothelial-cells-against-oxygen-glucose-deprivation-reoxygenation-injury-involvement-of-inhibiting-autophagy-via-class-i-pi3k-akt-mtor-signaling-pathway
#2
Guang Yang, Ning Wang, Sai Wang Seto, Dennis Chang, Huazheng Liang
The present study aimed to test whether Hydroxysafflor yellow A (HSYA) protects the brain microvascular endothelial cells (BMECs) injury induced by oxygen glucose deprivation/reoxygenation (OGD/R) via the PI3K/Akt/mTOR autophagy signaling pathway. Primary rat BMECs were cultured and identified by the expression of factor VIII-related antigen before being exposed to OGD/R to imitate ischemia/reperfusion (I/R) damage in vitro. The protective effect of HSYA was evaluated by assessing (1) cellular morphologic and ultrastructural changes; (2) cell viability and cytotoxicity; (3) transendothelial electrical resistance (TEER) of monolayer BMECs; (4) cell apoptosis; (5) fluorescence intensity of LC3B; (6) LC3 mRNA expression; (7) protein expressions of LC3, Beclin-1, Zonula occludens-1 (ZO-1), phospho-Akt (p-Akt), Akt, phospho-mTOR (p-mTOR) and mTOR...
May 15, 2018: Brain Research Bulletin
https://www.readbyqxmd.com/read/29774585/a-mild-form-of-haemophilia-a-is-associated-with-two-factor-viii-missense-mutations-in-german-fleckvieh-cattle
#3
Sina Reinartz, Christine Weiß, Reinhard Mischke, Ottmar Distl
No abstract text is available yet for this article.
May 18, 2018: Animal Genetics
https://www.readbyqxmd.com/read/29774375/-successful-treatment-of-acquired-hemophilia-a-with-recombinant-porcine-factor-viii
#4
K Trautmann-Grill, O Tiebel, K Hölig, U Platzbecker
Acquired hemophilia A is a rare, potentially life-threatening disease resulting from autoantibodies against coagulation factor VIII. We report the case of a patient with acquired hemophilia A and severe bleeding after incision of a peritonsillar abscess. Treatment with high dose factor VIII and recombinant activated factor VII failed to control bleeding. However, a single infusion of recombinant porcine factor VIII stopped bleeding efficiently and resulted in measurable factor VIII levels.
May 17, 2018: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/29769259/novel-therapeutics-for-hemophilia-and-other-bleeding-disorders
#5
Michael U Callaghan, Robert Sidonio, Steven W Pipe
Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment for these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase III trial in people with hemophilia A and inhibitors...
May 16, 2018: Blood
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
#6
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
#7
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/29767244/propranolol-induces-hemangioma-endothelial-cell-apoptosis-via-a-p53%C3%A2-bax-mediated-pathway
#8
Tian-Hua Yao, Parekejiang Pataer, Krishna Prasad Regmi, Xi-Wen Gu, Quan-Yan Li, Jing-Ting Du, Su-Meng Ge, Jun-Bo Tu
The use of propranolol for the treatment of infantile hemangioma (IH) has been widely investigated in recent years. However, the underlying therapeutic mechanism of propranolol for the treatment of IH remains poorly understood. The aim of the present study was to investigate the expression of proteins regulated by cellular tumor antigen p53 (p53) in associated apoptosis pathways in IH endothelial cells (HemECs) treated with propranolol. Furthermore, the present study aimed to investigate the exact apoptotic pathway underlying the therapeutic effect of propranolol against IH...
May 14, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29765291/the-potential-close-future-of-hemophilia-treatment-gene-therapy-tfpi-inhibition-antithrombin-silencing-and-mimicking-factor-viii-with-an-engineered-antibody
#9
REVIEW
Wolfgang Korte, Lukas Graf
Summary Hemophilia is one of the best researched monogenic diseases. Hemophilia A will affect approximately 1:5,000 male live births. In recent decades, great progress has been made with the introduction of recombinant proteins in the 1990s for therapy and prophylaxis, securing adequate availability and, with the introduction of the prophylaxis concept, reducing the negative impact of hemophilia on morbidity (especially arthropathy). Despite this progress, there are still challenges to overcome to secure adequate prophylaxis and treatment: for the time being, causal pharmacological hemophilia prophylaxis and therapy requires repeated i...
April 2018: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/29765290/extended-half-life-factor-viii-and-factor-ix-preparations
#10
REVIEW
Lukas Graf
In the last couple of years, several extended half-life factor VIII and factor IX preparations were intensively studied and gained approval. In order to extend half-lives, techniques like fusion to protein conjugates (Fc part of IgG1 or albumin), chemical modification (PEGylation), and protein sequence modification are implemented. With these techniques, it is possible to extend half-lives of factor IX products 4- to 6- fold, while half-life extension of factor VIII products is limited to 1.5- to 2-fold due to their interaction with von Willebrand factor...
April 2018: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/29762905/pharmacokinetics-and-the-transition-to-extended-half-life-factor-concentrates-communication-from-the-ssc-of-the-isth
#11
Margaret V Ragni, Stacy E Croteau, Massimo Morfini, Marjon H Cnossen, Alfonso Iorio
Extended half-life proteins (EHL) are increasingly used in clinical practice, but there is no standardized approach to sampling, interpretation, and implementation of pharmacokinetics (PK) data to maximize treatment benefit. The goal of EHL treatment is to attain a trough level sufficient to protect against spontaneous bleeds and reduce infusion frequency and limitations on individual activity and lifestyle. Performing classical PK assessments requires multiple blood samples which is burdensome for patients and providers...
May 15, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29761301/importance-of-initial-concentration-of-factor-viii-in-a-mechanistic-model-of-in-vitro-coagulation
#12
M Susree, M Anand
This computational study generates a hypothesis for the coagulation protein whose initial concentration greatly influences the course of coagulation. Many clinical malignancies of blood coagulation arise due to abnormal initial concentrations of coagulation factors. Sensitivity analysis of mechanistic models of blood coagulation is a convenient method to assess the effect of such abnormalities. Accordingly, the study presents sensitivity analysis, with respect to initial concentrations, of a recently developed mechanistic model of blood coagulation...
May 14, 2018: Acta Biotheoretica
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
#13
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/29750671/successful-treatment-of-severe-menorrhagia-at-menarche-with-recombinant-factor-viia-in-an-adolescent-girl-with-type-iii-von-willebrand-s-disease
#14
Dilek Gurlek Gokcebay, Vildan Culha, Nese Yarali, Namik Yasar Ozbek
: Type III von Willebrand's disease (vWD) is an inherited bleeding disorder, which is frequently associated with menorrhagia in women. Treatment options include antifibrinolytics, desmopressin, von Willebrand factor/factor VIII concentrates and in intractable bleeding circumstances recombinant factor VIIa (rFVIIa). We present an adolescent case with type III vWD who had a menorrhagia at menarche that was refractory to the standard treatment and ultimately was treated with rFVIIa successfully.
May 9, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29749544/repair-of-urethral-defects-by-an-adipose-mesenchymal-stem-cell%C3%A2-porous-silk-fibroin-material
#15
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/29745889/potentially-traumatic-events-ptsd-and-post-traumatic-stress-spectrum-in-patients-with-fibromyalgia
#16
Ciro Conversano, Claudia Carmassi, Carlo A Bertelloni, Laura Marchi, Tommaso Micheloni, Manuel G Carbone, Giovanni Pagni, Claudia Tagliarini, Gabriele Massimetti, Laura M Bazzichi, Liliana Dell'Osso
OBJECTIVES: Fibromyalgia (FM) is defined as a severe, chronic, non-articular rheumatic condition characterised by widespread musculoskeletal pain, hyperalgesia and generalised tender points, in the absence of inflammatory or structural musculoskeletal abnormalities. Pain is the predominant symptom, allodynia and hyperalgesia are common signs. Extreme fatigue, impaired cognition and non-restorative sleeping difficulties coexist in addition to other somatic symptoms. Several studies suggest there is a meaningful relationship between FM and the psychological symptoms of depression and post-traumatic stress disorder (PTSD)...
April 24, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#17
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743396/-congenital-factor-v-and-factor-viii-deficiency-discovered-in-an-elderly-patient-with-abnormal-bleeding-after-trauma
#18
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/29741692/the-challenges-of-the-family-physician-policy-in-iran-a-systematic-review-and-meta-synthesis-of-qualitative-researches
#19
Masoud Behzadifar, Meysam Behzadifar, Sanaz Heidarvand, Hasan Abolghasem Gorji, Aidin Aryankhesal, Sharare Taheri Moghadam, Roghayeh Mohammadibakhsh, Nicola Luigi Bragazzi
Background: A good level of health requires the establishment of primary health care. Family physician policy (FPP) is probably one of such initiatives, which enables societies attaining the universal health coverage. Objective: The present study is the first systematic review and meta-synthesis that seeks to provide a more comprehensive understanding of the challenges for FPP in Iran. Method: Several international scholarly databases (namely, ISI/Web of Sciences, PubMed/MEDLINE via Ovid, Embase, PsycINFO, Scopus and CINAHL via EBSCO), as well as three Iranian databases [MagIran, Irandoc and Scientific Information Database (SID) databases], were mined from January 2006 to December 2017...
May 7, 2018: Family Practice
https://www.readbyqxmd.com/read/29741261/new-challenges-and-best-practices-for-the-laboratory-monitoring-of-factor-viii-and-factor-ix-replacement
#20
REVIEW
D Van den Bossche, K Peerlinck, M Jacquemin
Several recombinant factor VIII and factor IX concentrates with extended half-life (EHL) have recently been validated by clinical studies. The availability of these novel concentrates is expected to significantly facilitate the treatment of patients with hemophilia A and B. However, the modification applied to these molecules has introduced variations in their activity measurement in routine coagulation assays. Depending on the assays, underestimations of up to 10-fold or overestimations of up to approximately 30-fold in the measurements of the recovery have been reported in some factor concentrates...
May 2018: International Journal of Laboratory Hematology
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