25,004 results match your criteria Factor VIII


The von Willebrand factor D'D3 assembly and structural principles for factor VIII binding and concatemer biogenesis.

Blood 2019 Jan 14. Epub 2019 Jan 14.

Children's Hospital Boston and Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA, United States;

D assemblies comprise half of von Willebrand factor yet are of unknown structure. D1 and D2 in the prodomain and D'D3 in mature VWF at Golgi pH form helical VWF tubules in Weibel Palade bodies and template dimerization of D3 through disulfides to form ultralong VWF concatemers. D'D3 forms the binding site for factor VIII. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2018-10-876300DOI Listing
January 2019
1 Read

Pharmacokinetic-guided dosing of factor VIII concentrate in a morbidly obese severe haemophilia A patient undergoing orthopaedic surgery.

BMJ Case Rep 2019 Jan 14;12(1). Epub 2019 Jan 14.

Department of Paediatric Haematology, Erasmus University Medical Center, Sophia Childrens Hospital Rotterdam, Rotterdam, The Netherlands.

A 58-year-old morbidly obese male (body mass index: 38 kg/m) with severe haemophilia A underwent total knee replacement surgery. Perioperatively, factor VIII (FVIII) levels were measured daily and maximum (MAP) Bayesian estimation was used to calculate the individual pharmacokinetic (PK) parameters and doses required to obtain prescribed FVIII target levels. In the MAP Bayesian procedure, a population PK model was used in which PK parameters were normalised using body weight. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2018-226812DOI Listing
January 2019

Prognostic value of uPAR expression and angiogenesis in primary and metastatic melanoma.

PLoS One 2019 14;14(1):e0210399. Epub 2019 Jan 14.

Centre for Cancer Biomarkers CCBIO, Department of Clinical Medicine, University of Bergen, Bergen, Norway.

Angiogenesis is important for the progression of cutaneous melanoma. Here, we analyzed the prognostic impact of the angiogenic factor urokinase plasminogen activator resecptor (uPAR), vascular proliferation index (VPI) and tumor necrosis as a measure of hypoxia in a patient series of nodular melanomas (n = 255) and matched loco-regional metastases (n = 78). Expression of uPAR was determined by immunohistochemistry and VPI was assessed by dual immunohistochemistry using Factor-VIII/Ki67 staining. Read More

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210399PLOS
January 2019

Individual differences of plasma proteins and factors in fresh frozen plasma from Chinese regional blood donors.

J Thromb Thrombolysis 2019 Jan 12. Epub 2019 Jan 12.

Department of Blood Transfusion, Chinese PLA General Hospital, No. 28 Fuxing Road, Haidian District, Beijing, China.

Currently, single fresh frozen plasma (FFP) for clinical use is derived from individual blood donors. The objective of this study is to investigate the differences in single FFP units to make related strategies for improving FFP transfusion efficacy. 120 units of single FFP were selected randomly from Chinese PLA Clinical Blood Transfusion Center in Beijing, China. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s11239-018-1789-9DOI Listing
January 2019

Bayesian pharmacokinetic-guided prophylaxis with recombinant factor VIII in severe or moderate haemophilia A.

Thromb Res 2019 Jan 3;174:151-162. Epub 2019 Jan 3.

Pharmacy Department, Drug Clinical Area, University and Polytechnic Hospital La Fe, Avda. Fernando Abril Martorell 106, 46026, Valencia, Spain.

Introduction: Personalised pharmacokinetics (PK) using Bayesian analysis with limited sampling is assumed to help to optimise prophylaxis in haemophilia A (HA) patients.

Materials And Methods: Our prospective, observational study analysed the influence of PK parameters on clinical variables (bleeding rates, joint status, adherence, and consumption) using myPKFiT® in a cohort of twenty-one severe and moderate HA patients on prophylaxis with recombinant FVIII (Advate®) in two periods of one year, the first before PK-based tailoring and the second after PK-guided prophylaxis. Intra-individual and inter-individual coefficients of variation (CV) of half-life (t) were calculated. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.thromres.2018.12.027DOI Listing
January 2019

Dose tailoring of human cell line-derived recombinant factor VIII simoctocog alfa: using a limited sampling strategy in patients with severe haemophilia A.

Br J Clin Pharmacol 2019 Jan 11. Epub 2019 Jan 11.

Unité d'Hémostase Clinique, Hôpital Cardiologique Louis Pradel, Université Lyon 1, Lyon, France.

Background: The use of www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2607 prophylaxis in haemophilia A is considered the standard of care, particularly in children. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bcp.13858DOI Listing
January 2019

How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors.

Semin Thromb Hemost 2019 Jan 10. Epub 2019 Jan 10.

Department of Haematology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.

The activated partial thromboplastin time (APTT) assay is a very common coagulation test, used for several reasons. The test is conventionally used for assessing the contact factor (intrinsic) pathway of blood coagulation, and thus for screening deficiencies in this pathway, most typically factors VIII, IX, and XI. The APTT is also sensitive to contact factor deficiencies, including factor XII, prekallikrein, and high-molecular-weight kininogen. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0038-1677018DOI Listing
January 2019

A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury.

Case Rep Dent 2018 9;2018:7185263. Epub 2018 Dec 9.

Department of Maxillofacial Surgery, Tokyo Medical and Dental University, Tokyo, Japan.

Background: Acquired hemophilia A (AHA) is a rare disorder which results from the presence of autoantibodies against blood coagulation factor VIII. The initial diagnosis is based on the detection of an isolated prolongation of the activated partial thromboplastin time (aPTT) with negative personal and family history of bleeding disorder. Definitive diagnosis is the identification of reduced FVIII levels with evidence of FVIII neutralizing activity. Read More

View Article

Download full-text PDF

Source
https://www.hindawi.com/journals/crid/2018/7185263/
Publisher Site
http://dx.doi.org/10.1155/2018/7185263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6304843PMC
December 2018
1 Read

Initiation of Pediatric Clinical Trials for Coagulation Factors: Application of Pharmacokinetics and Allometry to First-in-Pediatric Dose Selection.

Authors:
Iftekhar Mahmood

J Clin Pharmacol 2019 Jan 9. Epub 2019 Jan 9.

Office of Tissue & Advanced Therapies (OTAT), Center for Biologics Evaluation and Research, Food & Drug Administration, Silver Spring, MD, USA.

Allometric scaling is a method that can be used for the extrapolation of pharmacokinetic parameters from adults to children. Subsequently, these allometrically predicted PK parameters can be used to project a suitable dose to initiate a pediatric clinical trial. The objective of this study was to predict clearance and in vivo recovery of coagulation factors VIII and IX allometrically in children from 1 year of age to adolescents and then project the first-in-children dose. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/jcph.1371DOI Listing
January 2019

Red blood cells: the forgotten player in hemostasis and thrombosis.

J Thromb Haemost 2019 Jan 7. Epub 2019 Jan 7.

Department of Cell and Developmental Biology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.

New evidence has stirred up a long-standing but undeservedly forgotten interest in the role of erythrocytes, or red blood cells (RBCs), in blood clotting and its disorders. This review summarizes the most recent research that describes the involvement of RBCs in hemostasis and thrombosis. There are both quantitative and qualitative changes in RBCs that affect bleeding and thrombosis, as well as interactions of RBCs with cellular and molecular components of the hemostatic system. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jth.14360DOI Listing
January 2019

Emicizumab (ACE910): Clinical background and laboratory assessment of hemophilia A.

Adv Clin Chem 2019 16;88:151-167. Epub 2018 Nov 16.

Department of Haematology, Sydney Centres fosr Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research, NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia.

Congenital hemophilia A, a relatively common and sometimes life-threatening bleeding disorder, is caused by inherited deficiency of clotting factor (F) VIII. The adoption of an appropriate medical and environmental prophylaxis is critical for long-term management of hemophilia because it will considerably reduce the number of both mild and severe bleeding episodes. Among the many therapeutic options that have become available over the past decades, ACE910 (also known as emicizumab) is a bispecific immunoglobulin G antibody characterized by its unique ability to bind FIX or FIXa on one arm and FX on the other, thus abrogating FVIII activity in vivo. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/bs.acc.2018.10.003DOI Listing
November 2018

PCSK9 in Haemostasis and Thrombosis: Possible Pleiotropic Effects of PCSK9 Inhibitors in Cardiovascular Prevention.

Thromb Haemost 2019 Jan 3. Epub 2019 Jan 3.

Division of Internal and Cardiovascular Medicine, Department of Medicine, University of Perugia, Perugia, Italy.

Since increased cholesterol levels are crucial in determining the development of atheroma, their reduction represents a mainstay in primary and secondary cardiovascular prevention. The most recent spectacular advancement in cholesterol-lowering therapy is represented by proprotein convertase subtilisin/kexin type-9 (PCSK9) inhibitors. Although their benefit over currently available treatments has been ascribed primarily to their strong low-density lipoprotein (LDL)-cholesterol reducing action, several clues suggest that PCSK9 inhibitors may also influence platelet function and blood coagulation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0038-1676863DOI Listing
January 2019
1 Read

Treatment of acquired haemophilia with recombinant porcine factor VIII: A successful strategy in a patient with unstable angina.

Haemophilia 2019 Jan 3. Epub 2019 Jan 3.

Department of Haematology, Leeds Comprehensive Care Haemophilia Centre, St James's University Hospital, Leeds, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/hae.13644DOI Listing
January 2019

Inhibitor development, safety and efficacy of Advate among previously treated patients with hemophilia A in a postmarketing surveillance in Japan.

Int J Hematol 2019 Jan 2. Epub 2019 Jan 2.

Kitakyushu Yahata-Higashi Hospital, Kitakyusyu, Japan.

Rurioctocog alfa (recombinant factor VIII: Advate) is available for the control of bleeding in patients with hemophilia A in Japan. To evaluate the inhibitor development, safety, and efficacy of rurioctocog alfa, a non-interventional and observational postmarketing surveillance was conducted on 352 previously treated Japanese patients aged 1-76 years with ≥ 4 exposure days under the conditions of routine clinical practice. A post-hoc comparison of the mean annualized bleeding rates which required treatment with rurioctocog alfa detected a statistically significant difference (P < 0. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12185-018-02574-xDOI Listing
January 2019

Gastric Cancer, Hemophilia A and Angiodysplasia - An Unreported Association with Clinical Implications in a Patient with Digestive Hemorrhage.

Chirurgia (Bucur) 2018 Nov-Dec;113(6):857-866

Gastric cancer is a rare cause of upper digestive hemorrhage. Associated co-morbidities may have a detrimental effect on both early and long-term outcomes after surgery for gastric cancer. Association of gastric adenocarcinoma with hemophilia A and angiodysplasia was not previously reported, and the impact on postoperative outcome is not known. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.21614/chirurgia.113.6.857DOI Listing
December 2018

Dichloroacetate is an antimetabolite that antagonizes acetate and deprives cancer cells from its benefits: A novel evidence-based medical hypothesis.

Med Hypotheses 2019 Jan 22;122:206-209. Epub 2018 Nov 22.

Department of Physiology, Taibah College of Medicine, Taibah University, Al-Madinah Al-Munawwarah, Saudi Arabia.

Dichloroacetate (DCA) is a promising safe anticancer drug that cured a patient with chemoresistant non-Hodgkin's lymphoma and treated lactic acidosis effectively. The well-known mechanism of DCA action is through stimulating Krebs cycle (stimulating pyruvate dehydrogenase via inhibiting pyruvate dehydrogenase kinase). This prevents lactate formation (Warburg effect) depriving cancer cells of lactate-based benefits e. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.mehy.2018.11.012DOI Listing
January 2019
1 Read

[Effects of 37 centigrade volume resuscitation on coagulation function and blood lactic acid in neonates with septic shock].

Zhonghua Wei Zhong Bing Ji Jiu Yi Xue 2018 Dec;30(12):1146-1149

Department of Pediatrics, First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, Jiangxi, China (Zhong XM, Zhong M, Luo KY, Liao HQ, Wang HB); Department of Laboratory, First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, Jiangxi, China (Shen ZM); Department of Pediatrics, People's Hospital of Huichang County, Ganzhou 341000, Jiangxi, China (Tu Q). Corresponding author: Zhong Xiaoming, Email:

Objective: To investigate the effect of volume resuscitation with normal saline (NS) at 37 centigrade on the coagulation function and microcirculation of neonates with septic shock.

Methods: Children with septic shock admitted to neonatal intensive care unit (NICU) of the First Affiliated Hospital of Gannan Medical University were enrolled. Twenty-four newborns with septic shock were divided into two groups by random number table method (12 in each group), and were resuscitated with 10 mL/kg at 25 centigrade NS and 37 centigrade NS respectively on the basis of routine treatment. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.2095-4352.2018.012.009DOI Listing
December 2018

Coagulation failure is associated with bleeding events and clinical outcome during systemic inflammatory response and sepsis in ACLF: an observational cohort study.

Liver Int 2018 Dec 27. Epub 2018 Dec 27.

Departments of Hepatology, Institute of Liver and Biliary Sciences, D1 Vasant Kunj, New Delhi, 110070.

Background: Patients with acute-on-chronic liver failure (ACLF) have coagulation failure in the setting of systemic inflammatory syndrome (SIRS), sepsis and extra-hepatic organ failures.

Methods: Consecutive ACLF patients without sepsis at baseline, were assessed at days 0, 3, and 7 with thromboelastography (TEG) and specific assays [factor VIII, vonWillebrand factor (vWF), protein C and antithrombin III (ATIII)] and followed for development of sepsis, bleeding and outcome.

Results: Of 243 patients, 114 [63% ethanol related; mean age 44. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/liv.14034DOI Listing
December 2018
4.850 Impact Factor

Accurate measurement of extended half-life and unmodified factor VIII low levels with one-stage FVIII assays is dependent on the matrix of calibration curves.

Haemophilia 2018 Dec 27. Epub 2018 Dec 27.

Clinical Department of Laboratory Medicine, University Hospitals of Leuven, Leuven, Belgium.

Introduction: The monitoring of factor VIII (FVIII) replacement therapy relies on the accurate measurement of FVIII activity over a large concentration range. However, unexplained overestimation of low FVIII levels has recently been reported with extended half-life recombinant FVIIIs.

Aim: The objective of this study was to confirm previous publications indicating that the reagents used to generate the calibration curves determine the accuracy of the measurement of low FVIII levels. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/hae.13656DOI Listing
December 2018

Genome-Wide Association Trans-Ethnic Meta-Analyses Identifies Novel Associations Regulating Coagulation Factor VIII and von Willebrand Factor Plasma Levels.

Circulation 2018 Nov 20. Epub 2018 Nov 20.

Epidemiology, University of Washington, Seattle, UNITED STATES.

Background: Factor VIII (FVIII) and its carrier protein von Willebrand factor (VWF) are associated with risk of arterial and venous thrombosis and with hemorrhagic disorders. We aimed to identify and functionally test novel genetic associations regulating plasma FVIII and VWF.

Methods: We meta-analyzed genome-wide association results from 46,354 individuals of European, African, East Asian, and Hispanic ancestry. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCULATIONAHA.118.034532DOI Listing
November 2018
2 Reads
14.430 Impact Factor

Thrombin generation assay for testing hemostatic effect of factor VIII concentrates in patients with hemophilia A and inhibitors: In vitro results from the PredicTGA study.

Thromb Res 2018 Dec 6;174:84-87. Epub 2018 Dec 6.

IRCCS Cà Granda Maggiore Policlinico Hospital Foundation, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milano, Italy.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.thromres.2018.12.007DOI Listing
December 2018
1 Read

Crosstalk Between Glucocorticoid Receptor and Early-growth Response Protein 1 Accounts for Repression of Brain-derived Neurotrophic Factor Transcript 4 Expression.

Neuroscience 2018 Dec 19;399:12-27. Epub 2018 Dec 19.

Inserm 1185, Fac Med Paris Sud, Université Paris-Saclay, Le Kremlin-Bicêtre, France. Electronic address:

The brain-derived neurotrophic factor (BDNF) is a key player in brain functions such as synaptic plasticity, stress, and behavior. Its gene structure in rodents contains 8 untranslated exons (I to VIII) whose expression is finely regulated and which spliced onto a common and unique translated exon IX. Altered Bdnf expression is associated with many pathologies such as depression, Alzheimer's disease and addiction. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuroscience.2018.12.012DOI Listing
December 2018
1 Read

Eptacog beta: a novel recombinant human factor VIIa for the treatment of hemophilia A and B with inhibitors.

Expert Rev Hematol 2018 Dec 21. Epub 2018 Dec 21.

a Haemophilia Treatment Centre, University Hospital Montpellier , France .

Introduction: Hemophilia A and B are X-linked recessive disorders caused by the deficiency of factor VIII or factor IX, respectively. Bleeding episodes are treated with factor replacement therapy. The most serious complication of this treatment is the development of inhibitors. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/17474086.2019.1560259DOI Listing
December 2018
1 Read

Acquired Hemophilia A Presenting as Intramuscular Hematoma.

J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709618817572. Epub 2018 Dec 6.

MedStar Washington Hospital Center, Washington, DC, USA.

Acquired hemophilia A poses a clinical and diagnostic challenge. Although rare, it is still the most common acquired factor deficiency. We present a case of acquired hemophilia A diagnosed in a 71-year-old female who presented with a right thigh hematoma of acute onset. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2324709618817572DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299309PMC
December 2018

Oral surgery for combined haemophilia A and B. The dos and don'ts presented in a clinical scenario.

Ann Ital Chir 2018 ;89:374-378

Background: Haemophilia A (factor VIII deficiency), B (factor IX deficiency) and C (factor XI deficiency) are common genetic bleeding disorders. Most often they are caused by the absence or defective function of coagulation factors, causing inefficient blood clots.

Case Report: The present manuscript describes a rare case of a combined haemophilia A and B patient, who underwent several extractions. Read More

View Article

Download full-text PDF

Source
January 2018
2 Reads

Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency.

J Blood Med 2018 29;9:227-239. Epub 2018 Nov 29.

Clinical, Medical and Regulatory Affairs, Novo Nordisk Inc., Plainsboro, NJ, USA.

Introduction: Continuous infusion (CI) of clotting factors as a replacement therapy for perioperative hemostatic protection has been performed for many years, including with factors VIII and IX and recombinant activated factor VII (rFVIIa). This approach provides steady factor levels without requiring frequent administration of bolus doses.

Aim: To review safety, efficacy, and dosing data regarding CI of rFVIIa for hemostatic management of patients with congenital hemophilia with inhibitors (CHwI) or congenital factor VII deficiency (C7D). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2147/JBM.S184040DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276614PMC
November 2018
1 Read

[A Pediatric Case of Acquired Hemophilia A: The Usefulness of the Activated Partial Thromboplastin Time (APTT) Cross-Mixing Test for Early Diagnosis].

J UOEH 2018;40(4):331-337

Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan.

Acquired hemophilia A (AHA), a bleeding disorder caused by autoantibodies against FVIII, has the potential for life-threatening bleeding. The annual onset rate is said to be one in 4 million people, but diagnosis examples increase in adults because a disorder concept penetrated. AHA is quite rare in children, with an incidence rate of 0. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7888/juoeh.40.331DOI Listing
January 2018
1 Read

Anaphylaxis after treatment with recombinant factor VIII: investigation and therapeutic challenge.

BMJ Case Rep 2018 Dec 14;11(1). Epub 2018 Dec 14.

Application Lab, R&D Department, Roxall Group, Bilbao, Spain.

We report a 10-year-old patient with haemophilia A developing anaphylaxis to recombinant factor VIII (octocog alfa). Allergic reactions, and especially anaphylactic events, are rare in patients with haemophilia A. The nature of these reactions is not fully understood. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2018-227426DOI Listing
December 2018
1 Read

Effects of attachment to and participation in the community on motivation to participate in dementia prevention and support activities: analysis of Web survey data.

Psychogeriatrics 2018 Nov 22. Epub 2018 Nov 22.

Education and Innovation Center, National Center for Geriatrics and Gerontology, Aichi, Japan.

Aim: The objective of this study was to elucidate the effects of a sense of attachment to the community (attachment) and participation in community activities (participation) on motivation to participate in dementia prevention and support activities (motivation).

Methods: We conducted a nationwide Web-based questionnaire survey of 1693 men and women aged ≥20 years. The questionnaire items used in the analysis asked about the following: (i) demographic characteristics (i. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/psyg.12388DOI Listing
November 2018
1 Read

[Systemic lupus erythematosus with acquired hemophilia A: a case report].

Authors:
F Yang Y S Zhou Y Jia

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1108-1111

Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. Read More

View Article

Download full-text PDF

Source
December 2018
5 Reads

New therapies using non-factor products for patients with hemophilia and inhibitors.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Regular prophylaxis with factor (F)VIII or FIX products to prevent bleeding in patients with severe hemophilia (H)A and HB, respectively, results in marked suppressions of the onset of arthropathy, and contributes greatly to improvements in quality of life. Some issues remain with the use of clotting factor replacement therapy, however. The need for multiple intravenous administrations is associated with a substantial mental and physical burden, and the hemostatic effect of bypassing agents (BPAs) in patients with inhibitor is inconsistent. Read More

View Article

Download full-text PDF

Source
http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-07
Publisher Site
http://dx.doi.org/10.1182/blood-2018-07-820712DOI Listing
December 2018
6 Reads

Treatment of rare factor deficiencies other than hemophilia.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Universita degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy

The deficiency of fibrinogen, prothrombin, factor V, VII, VIII, IX, X, XI and XIII, called rare coagulation disorders (RCDs), may result in coagulopathies leading to spontaneous or post-trauma and post-surgery hemorrhages. RCDs are characterized by a wide variety of symptoms, from mild to severe, which can vary significantly from one disease to another and from one patient to another. The most typical symptoms of all RCDs are mucosal bleedings and bleeding at the time of invasive procedures, while other life-threatening symptoms such as central nervous system bleeding and hemarthroses are mostly present only in some deficiencies (afibrinogenemia, FX and FXIII). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2018-06-820738DOI Listing
December 2018
1 Read

Update on clinical gene therapy for hemophilia.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Wells Center for Pediatric Research, Indiana University, Indianapolis, IN, United States.

In contrast to other diverse therapies for the X-linked bleeding disorder hemophilia that are currently in clinical development, gene therapy holds the promise of a lasting cure with a single drug administration. Near to complete correction of hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) has now been achieved in patients by hepatic in vivo gene transfer. Adeno-associated viral (AAV) vectors with different viral capsids and engineered to express high-levels and in some cases hyperactive coagulation factors were employed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2018-07-820720DOI Listing
December 2018
1 Read

Molecular cloning and expression analysis of coagulation factor VIII and plasminogen involved in immune response to GCRV, and immunity activity comparison of grass carp Ctenopharyngodon idella with different viral resistance.

Fish Shellfish Immunol 2018 Dec 14;86:794-804. Epub 2018 Dec 14.

Hunan Engineering Technology Research Center of Featured Aquatic Resources Utilization, Hunan Agricultural University, Changsha, 410128, China; Collaborative Innovation Center for Efficient and Health Production of Fisheries in Hunan Province, Changde, Hunan, 415000, China. Electronic address:

The grass carp reovirus (GCRV) has been shown to cause lethal infections in the grass carp Ctenopharyngodon idella (C. idella). In order to investigate the immune response to GCRV infection, the full-length cDNA sequences of coagulation factor VIII (CiFVIII) and plasminogen (CiPLG) from C. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.fsi.2018.12.024DOI Listing
December 2018
1 Read

Clotting and chromogenic factor VIII assay variability in post-infusion and spiked samples containing full-length recombinant FVIII or recombinant factor VIII Fc fusion protein (rFVIIIFc).

Int J Lab Hematol 2018 Dec 17. Epub 2018 Dec 17.

UK NEQAS Blood Coagulation, Sheffield, UK.

Introduction: Variability in FVIII measurement is a recognized problem. There are limited data for samples containing recombinant Factor VIII Fc fusion protein (rFVIIIFc). Many studies use samples for which factor concentrate has been spiked into FVIII deficient plasma in vitro. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ijlh.12940DOI Listing
December 2018

Inhibitor incidence in haemophilia A under exclusive use of a third-generation recombinant factor VIII concentrate: results of the HEMFIL Cohort Study.

Br J Haematol 2018 Dec 17. Epub 2018 Dec 17.

Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.15728DOI Listing
December 2018

Transcription factor TFAP2B up-regulates human corneal endothelial cell-specific genes during corneal development and maintenance.

J Biol Chem 2018 Dec 14. Epub 2018 Dec 14.

Osaka University Graduate School of Medicine, Japan.

The corneal endothelium, which originates from the neural crest via the periocular mesenchyme (PM), is crucial for maintaining corneal transparency. The development of corneal endothelial cells (CECs) from the neural crest is accompanied by the expression of several transcription factors, but the contribution of some of these transcriptional regulators to CEC development is incompletely understood. Here, we focused on activating enhancer-binding protein 2 (TFAP2, AP-2), a neural crest-expressed transcription factor. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1074/jbc.RA118.005527DOI Listing
December 2018

The milk thistle (Silybum marianum) compound Silibinin stimulates leukopoiesis from mouse embryonic stem cells.

Phytother Res 2018 Dec 11. Epub 2018 Dec 11.

Department of Physiology, Faculty of Medicine, Justus Liebig University Giessen, Giessen, Germany.

The milk thistle compound Silibinin (i.e., a 1:1 mixture of Silybin A and Silybin B) stimulates vasculogenesis of mouse embryonic stem (ES) cells. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ptr.6241DOI Listing
December 2018

Influence of canine donor plasma hemostatic protein concentration on quality of cryoprecipitate.

J Vet Intern Med 2019 Jan 11;33(1):124-131. Epub 2018 Dec 11.

Department of Clinical Sciences and Advanced Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Cryoprecipitate (CRYO) is a plasma component containing high concentrations of factor VIII (FVIII), von Willebrand factor (VWF), and fibrinogen. Because Greyhounds are reported to have lower plasma VWF and fibrinogen concentrations, their plasma may not yield high potency CRYO.

Objectives: To determine if plasma hemostatic protein concentration is a good predictor of CRYO potency and if a difference exists in quality of CRYO prepared from Greyhounds versus non-Greyhounds. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jvim.15376DOI Listing
January 2019

Prevention of the anti-factor VIII memory B-cell response by inhibition of the Bruton's tyrosine kinase in experimental hemophilia A.

Haematologica 2018 Dec 13. Epub 2018 Dec 13.

INSERM UMR S 1138, Centre de Recherche des Cordeliers, Paris, France;

Hemophilia A is a rare hemorrhagic disorder due to the lack of functional pro-coagulant factor VIII. Factor VIII replacement therapy in patients with severe hemophilia A results in the development of inhibitory anti-factor VIII IgG in up to 30% of the cases. To date, immune tolerance induction upon daily injection of large amounts of factor VIII is the only strategy to eradicate factor VIII inhibitors. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3324/haematol.2018.200279DOI Listing
December 2018
7 Reads

Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A.

Authors:
Paul Knöbl

Drugs 2018 Dec;78(18):1861-1872

Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies inhibiting the function of coagulation factor VIII. It is characterized by spontaneous bleeding in patients with no previous family or personal history of bleeding. Although several large registries have collected clinical data on AHA, limited information is available on the optimal management of AHA because controlled clinical trials are lacking. Read More

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s40265-018-1027-y
Publisher Site
http://dx.doi.org/10.1007/s40265-018-1027-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6294818PMC
December 2018
5 Reads

Effects of permanent cardiac pacemaker implantation on vascular endothelial function, blood coagulation and cardiac function in patients with bradycardia.

Exp Ther Med 2018 Dec 28;16(6):4717-4721. Epub 2018 Sep 28.

The Heart Center, Jining First People's Hospital, Jining, Shandong 272011, P.R. China.

Changes in vascular endothelial function, blood coagulation and cardiac function indexes after the implantation of a permanent cardiac pacemaker in patients with bradycardia were investigated. A total of 53 healthy people and 117 patients with bradycardia in Jining First People's Hospital from January 2015 to August 2017 were selected. Factor VIII: coagulation (FVIII:C), von Willebr and factor (vWF), antithrombin activity (AT:A), D-dimmer (D-D), thrombomodulin (TM), tissue factor (TF), left ventricular ejection fraction (LVEF) and left ventricular end-systolic volume (LVESV) in the non-pacemaker group and the pacemaker group were significantly different from those in the control group (P<0. Read More

View Article

Download full-text PDF

Source
http://www.spandidos-publications.com/10.3892/etm.2018.6808
Publisher Site
http://dx.doi.org/10.3892/etm.2018.6808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257567PMC
December 2018
9 Reads
0.941 Impact Factor

Factor VIII with 237 amino acids B-domain has extended half-life in F8-KO mice.

J Thromb Haemost 2018 Dec 7. Epub 2018 Dec 7.

Novo Nordisk Park, Måløv, Denmark.

Background: Factor VIII (FVIII) consists of the A1-A2-B-A3-C1-C2 domains. FVIII with an intermediate length B-domain of 226 amino acids (aa) has previously been evaluated in murine gene therapy studies.

Objective: To characterize FVIII with intermediate length B-domains in vitro and in vivo in F8-KO mice. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jth.14355DOI Listing
December 2018
1 Read

Management of Acquired Hemophilia A in Elderly Patients.

Case Rep Hematol 2018 13;2018:6757345. Epub 2018 Nov 13.

Division of Hematology, Takasago-Seibu Hospital, Takasago 676-0812, Japan.

This report describes six elderly patients with acquired hemophilia A (AHA), including four individuals aged ≥90 years. Bleeding symptoms were subcutaneous or intramuscular hemorrhage (=4), hematuria (=1), and hemorrhagic shock after tooth extraction (=1). Factor VIII (FVIII) activity ranged from <1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/6757345DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260550PMC
November 2018

The impact of blood type on transfusion after major emergency abdominal surgery.

Transfusion 2019 Jan 10;59(1):125-133. Epub 2018 Dec 10.

Center for Surgical Science (CSS), Department of Surgery, Zealand University Hospital, Køge & University of Copenhagen, København, Denmark.

Background: ABO dependent variation in von Willebrand factor (vWF) and factor VIII have an impact on hemostasis. Several contradictory studies have investigated the influence of ABO blood type in surgical hemorrhage. Emergency surgery is associated with an increased risk of perioperative hemorrhage leading to inferior clinical outcomes. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/trf.15067DOI Listing
January 2019
1 Read

Secondary inappropriate polycythemia with splenic hemangiosarcoma in a young adult cat.

Can Vet J 2018 Dec;59(12):1320-1324

Chungnam National University - Veterinary Internal Medicine, 99 Daehakro, gung dong Yu-seong gu, Deajeon 305-764, Republic of Korea (Seo, Hong, An); IDEXX Laboratories Ringgold standard institution, Westbrook, Maine, USA (Lee); University of California Davis School of Veterinary Medicine, Ringgold standard institution, 2112 Tupper Hall, One Shields Avenue, Davis, California 95616, USA (Rebhun).

A 20-month-old castrated male Korean shorthair cat was presented with a 3-week history of intermittent vomiting and anorexia, absolute erythrocytosis, and elevated erythropoietin levels. A diagnosis of splenic hemangiosarcoma was made by histopathology and immunohistochemical identification of factor VIII. Paraneoplastic erythrocytosis caused by a splenic hemangiosarcoma in a cat is described. Read More

View Article

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237254PMC
December 2018
1 Read

Evaluation of EC50 of factor VIII as predictor of prophylaxis efficacy in patients with severe haemophilia A.

Eur J Pharm Sci 2019 Feb 7;128:215-221. Epub 2018 Dec 7.

La Paz University Hospital, Haematology and Haemotherapy Department, Madrid, Spain. Electronic address:

Trough factor (F) VIII level is a not reliable bleeding risk indicator to predict prophylaxis efficacy in severe haemophilia A (SHA), therefore, accurate biomarkers are much needed. Thrombelastography (TEG) monitors both thrombin and clot formation addressing the global haemostatic status but its usefulness to tailor prophylaxis in haemophilia has been poorly evaluated. In this study, correspondence between individual pharmacodynamic/pharmacokinetic profile of FVIII and joint condition, physical activity and bleeding phenotype of SHA patients under prophylactic treatment was assessed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejps.2018.12.003DOI Listing
February 2019

Test-retest properties of the Patient Reported Outcomes, Burdens and Experiences (PROBE) questionnaire and its constituent domains.

Haemophilia 2018 Dec 6. Epub 2018 Dec 6.

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Background: The Patient Reported Outcomes, Burdens and Experiences (PROBE) study aims to develop and validate questionnaire for assessing health status in patients with haemophilia and participants without bleeding disorders.

Objective: To investigate the test-retest properties of the PROBE questionnaire.

Methods: The PROBE questionnaire covers four domains and is comprised of 29 questions. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/hae.13649DOI Listing
December 2018
1 Read

VIII. CONCLUSION: CO-CONSTRUCTING A SECURE BASE PARTNERSHIP: MOTHER-CHILD INTERACTIONS, COMMUNICATION, AND SCRIPT REPRESENTATIONS.

Monogr Soc Res Child Dev 2018 12;83(4):121-134

Using the secure base construct, the evidence presented indicates that interactional experience continues to be a central factor in the organization of mother-child attachment relationships. The parent-child codetermination process that establishes their relationship in infancy expands during the preschool years. Furthermore, with the increasingly relevant role of language, parent-child verbal communication during this time plays an important part in structuring children's attachment behavior and knowledge. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/mono.12395DOI Listing
December 2018
2 Reads