25,176 results match your criteria Factor VIII


Changes in hemostasis parameters in nonfatal methicillin-sensitive Staphylococcus aureus bacteremia complicated by endocarditis or thromboembolic events: A prospective age-gender adjusted cohort study.

APMIS 2019 Apr 22. Epub 2019 Apr 22.

Division of Infectious Diseases, Inflammation Center, University of Helsinki and Helsinki University Hospital, Finland.

The aim was to examine changes in hemostasis parameters in endocarditis and thromboembolic events in nonfatal methicillin-sensitive Staphylococcus aureus bacteremia (MS-SAB) - a topic not evaluated previously. 155 patients were recruited and categorized according to presence of endocarditis or thromboembolic events with gender-age adjusted controls. Patients who deceased or patients not chosen as controls were excluded. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/apm.12955
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http://dx.doi.org/10.1111/apm.12955DOI Listing
April 2019
1 Read

Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study.

Lancet Haematol 2019 Apr 16. Epub 2019 Apr 16.

Hospital Universitario La Paz, Autónoma University, Madrid, Spain.

Background: Emicizumab, a subcutaneously administered, humanised, bispecific, monoclonal antibody, is approved to treat people with haemophilia A of all ages with and without coagulation factor VIII (FVIII) inhibitors. HAVEN 4 assessed emicizumab prophylaxis administered as one dose every 4 weeks in adults and adolescents with haemophilia A, regardless of FVIII inhibitor status.

Methods: In this phase 3, multicentre, open-label, two-stage study, patients aged 12 years and older with severe congenital haemophilia A (<1% of normal FVIII activity in blood) or haemophilia A with FVIII inhibitors, undergoing treatment with either FVIII concentrates or bypassing agents were recruited from three sites in Japan and Spain for a run-in cohort, and from 17 sites in Australia, Belgium, Japan, Poland, Spain, and the USA for a subsequent expansion cohort. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23523026193005
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http://dx.doi.org/10.1016/S2352-3026(19)30054-7DOI Listing
April 2019
1 Read

Single-molecule nanomechanical spectroscopy shows calcium ions contribute to chain association and structural flexibility of blood clotting factor VIII.

Biochem Biophys Res Commun 2019 Apr 16. Epub 2019 Apr 16.

CAS Key Laboratory of Interfacial Physics and Technology, Shanghai Institute of Applied Physics, Chinese Academy of Sciences, Shanghai, 201800, China; ZhangJiang Lab, Shanghai Synchrotron Radiation Facility, Shanghai Advanced Research Institute, Chinese Academy of Sciences, Shanghai, 201210, China. Electronic address:

Blood coagulation factor VIII (FVIII) can bind calcium ions and ion-protein interactions appear central importance for both their structure and function in coagulation cascade. However, the mechanism and details of how calcium dependent structure change of proteins to fulfill their function remain to be fully defined. In this work, PeakForce Quantitative Nanomechanics (PF-QNM) mode atomic force microscopy (AFM) was used to map the topography and mechanical properties of FVIII with single protein resolution under different calcium concentrations. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.04.068DOI Listing
April 2019
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Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions.

Cochrane Database Syst Rev 2019 Apr 19;4:CD011385. Epub 2019 Apr 19.

Van Creveldkliniek / Department of Haematology, University Medical Centre Utrecht, Heidelberglaan 100, Utrecht, Netherlands, 3584CT.

Background: Minor oral surgery or dental extractions (oral or dental procedures) are widely performed and can be complicated by hazardous oral bleeding, especially in people with an inherited bleeding disorder such as haemophilia or Von Willebrand disease (VWD). The amount and severity of singular bleedings depend on disease-related factors, such as the severity of the haemophilia, both local and systemic patient factors (such as periodontal inflammation, vasculopathy or platelet dysfunction) and intervention-related factors (such as the type and number of teeth extracted or the dimension of the wound surface). Similar to local haemostatic measures and suturing, antifibrinolytic therapy is a cheap, safe and potentially effective treatment to prevent bleeding complications in individuals with bleeding disorders undergoing oral or dental procedures. Read More

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http://doi.wiley.com/10.1002/14651858.CD011385.pub3
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http://dx.doi.org/10.1002/14651858.CD011385.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6474399PMC
April 2019
1 Read

Surgical Risk on Patients with Coagulopathies: Guidelines on Hemophiliac Patients for Oro-Maxillofacial Surgery.

Int J Environ Res Public Health 2019 Apr 17;16(8). Epub 2019 Apr 17.

Department of Biomedical and Dental Sciences and Morphological and Functional Imaging, Messina University, 98100 Messina, Italy.

Background: Haemophilia is a disease of genetic origin, which causes a defect in blood coagulation. Under normal conditions, in the case of leakage from the blood vessels, the blood forms a clot that reduces or blocks the bleeding. This process involves the activation of several plasma proteins in a cascade-like species. Read More

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https://www.mdpi.com/1660-4601/16/8/1386
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http://dx.doi.org/10.3390/ijerph16081386DOI Listing
April 2019
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In silico and in vitro evaluation of the impact of mutations in non-severe haemophilia A patients on assay discrepancies.

Ann Hematol 2019 Apr 17. Epub 2019 Apr 17.

Institute of Experimental Hematology and Transfusion Medicine, University of Bonn, Sigmund-Freud str. 25, 53127, Bonn, Germany.

Haemophilia A (HA) is caused by a lack or reduced amount of factor VIII protein (FVIII). About one-third of patients with non-severe HA carrying specific missense mutations show discrepant results between FVIII activity (FVIII:C), measured by one-stage or chromogenic two-stage assays. The aim of this study was to elucidate the mechanism underlying the assay discrepancy in vitro and in silico. Read More

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http://link.springer.com/10.1007/s00277-019-03691-1
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http://dx.doi.org/10.1007/s00277-019-03691-1DOI Listing
April 2019
2 Reads

Clinical practice of personalized prophylaxis in hemophilia: Illustrations of experiences and benefits from two continents.

Clin Case Rep 2019 Apr 20;7(4):689-694. Epub 2019 Feb 20.

Hemostasis and Thrombosis Unit, Division of Hematology Cliniques Universitaires Saint Luc, Université Catholique de Louvain Brussels Belgium.

These cases of people with hemophilia (PWH) illustrate the importance of understanding the patient's expectations and desires and adapting treatment to meet these needs, in addition to traditional clinical targets. Population PK modeling and FVIII products with improved PK profiles provide the opportunity to individualize care and improve long-term outcomes. Read More

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http://dx.doi.org/10.1002/ccr3.2021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452482PMC
April 2019
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Management of Odontogenic Fascial Space Infection in Hemophilia Patients: A Proposed Protocol.

J Maxillofac Oral Surg 2019 Jun 9;18(2):197-202. Epub 2018 May 9.

Department of Oral and Maxillofacial Surgery, Aligarh Muslim University, Aligarh, Uttar Pradesh 202002 India.

Purpose: The purpose of this study was to suggest a protocol for the management of odontogenic space infections in patients with hemophilia which is often incompletely addressed by clinicians because of the fear of excessive bleeding leading to prolonged morbidity.

Methods And Results: Six diagnosed cases of hemophilia (four cases of hemophilia A and two cases of hemophilia B) with odontogenic fascial space infection were included in the study. Apart from routine investigations, assessments were also done to identify inhibitors to factor VIII/IX. Read More

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http://dx.doi.org/10.1007/s12663-018-1117-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6441428PMC
June 2019
2 Reads

Restoration of FVIII expression by targeted gene insertion in the FVIII locus in hemophilia A patient-derived iPSCs.

Exp Mol Med 2019 Apr 17;51(4):45. Epub 2019 Apr 17.

Department of Physiology, Yonsei University College of Medicine, 50-1 Yonsei-ro Seodaemun-gu, Seoul, 03722, Korea.

Target-specific genome editing, using engineered nucleases zinc finger nuclease (ZFN), transcription activator-like effector nuclease (TALEN), and type II clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated protein 9 (Cas9), is considered a promising approach to correct disease-causing mutations in various human diseases. In particular, hemophilia A can be considered an ideal target for gene modification via engineered nucleases because it is a monogenic disease caused by a mutation in coagulation factor VIII (FVIII), and a mild restoration of FVIII levels in plasma can prevent disease symptoms in patients with severe hemophilia A. In this study, we describe a universal genome correction strategy to restore FVIII expression in induced pluripotent stem cells (iPSCs) derived from a patient with hemophilia A by the human elongation factor 1 alpha (EF1α)-mediated normal FVIII gene expression in the FVIII locus of the patient. Read More

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http://dx.doi.org/10.1038/s12276-019-0243-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6470126PMC
April 2019
1 Read
3.446 Impact Factor

Regional variation and cost implications of prescribed extended half-life factor concentrates among U.S. Haemophilia Treatment Centres for patients with moderate and severe haemophilia.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

St. Jude Children's Research Hospital, Memphis, Tennessee.

Background: Extended half-life (EHL) factor VIII (FVIII) and IX (FIX) products are intended to decrease the burden of prophylaxis for patients with haemophilia A or B. Whether these newer concentrates have led to meaningful clinical practice change remains vague.

Aim: To characterize the longitudinal use of standard (SHL) and EHL factor concentrates at haemophilia treatment centres (HTCs), using the ATHNdataset, a US database of 138 ATHN-affiliated HTCs. Read More

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http://dx.doi.org/10.1111/hae.13758DOI Listing
April 2019
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Efficacy and safety of prophylaxis with BAY 81-8973 in Chinese patients with severe haemophilia A enrolled in the LEOPOLD II trial.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Bayer, Whippany, New Jersey.

Introduction: BAY 81-8973 (Kovaltry ) is a full-length, unmodified recombinant human factor VIII approved in China for prophylaxis and on-demand treatment in patients with haemophilia A. Limited access to FVIII prophylaxis in China has historically led to this population being undertreated. This subanalysis of LEOPOLD II investigated whether the efficacy and safety of BAY 81-8973 varied between Chinese and non-Chinese patients. Read More

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http://dx.doi.org/10.1111/hae.13751DOI Listing
April 2019
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Undifferentiated spermatogonia regulate expression through NOTCH signaling and drive germ cell differentiation.

FASEB J 2019 Apr 16:fj201802361R. Epub 2019 Apr 16.

Department of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Cytochrome P450 family 26 subfamily B member 1 (CYP26B1) regulates the concentration of all-trans retinoic acid (RA) and plays a key role in germ cell differentiation by controlling local distribution of RA. The mechanisms regulating expression in postnatal Sertoli cells, the main components of the stem cell niche, are so far unknown. During gonad development, expression of is maintained by Steroidogenic Factor 1 (SF-1) and Sex-Determining Region Y Box-9 (SOX9), which ensure that RA is degraded and germ cell differentiation is blocked. Read More

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http://dx.doi.org/10.1096/fj.201802361RDOI Listing
April 2019
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Consensus statements on vaccination in patients with haemophilia-Results from the Italian haemophilia and vaccinations (HEVA) project.

Haemophilia 2019 Apr 16. Epub 2019 Apr 16.

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

Vaccination against communicable diseases is crucial for disease prevention, but this practice poses challenges to healthcare professionals in patients with haemophilia. Poor knowledge of the vaccination requirements for these patients and safety concerns often result in vaccination delay or avoidance. In order to address this issue, a panel of 11 Italian haemophilia and immunization experts conducted a Delphi consensus process to identify the main concerns regarding the safe use of vaccines in patients with haemophilia. Read More

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http://dx.doi.org/10.1111/hae.13756DOI Listing
April 2019
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Deep dermal and subcutaneous canine hemangiosarcoma in the perianal area: diagnosis of perianal mass in a dog.

Authors:
Eun Wha Choi

BMC Vet Res 2019 Apr 15;15(1):115. Epub 2019 Apr 15.

Department of Veterinary Clinical Pathology, College of Veterinary Medicine & Institute of Veterinary Science, Kangwon National University, 1 Kangwondaehak-gil, Chuncheon, Gangwon-do, 24341, Republic of Korea.

Background: Tumors of the perianal area occur frequently in dogs, and the two most common tumors are perianal gland adenoma and anal sac adenocarcinoma; others such as mast cell tumor, lymphoma and melanoma can also occur at this site. Diagnostic cytology is a useful technique and is usually used to establish a definitive diagnosis of some tumors in veterinary medicine. This report describes an extremely rare case of a deep dermal and subcutaneous canine hemangiosarcoma in the perianal area. Read More

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http://dx.doi.org/10.1186/s12917-019-1852-6DOI Listing
April 2019
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Hemostatic characteristics of thawed, pooled cryoprecipitate stored for 35 days at refrigerated and room temperatures.

Transfusion 2019 Apr;59(S2):1560-1567

Blood Research Program, US Army Institute of Surgical Research, Fort Sam Houston, Texas.

Background: Cryoprecipitate's shelf life is limited due to concerns over decreased clotting factor activity and contamination with extended storage. Hemostatic characteristics of thawed cryoprecipitate stored up to 35 days at refrigerated and room temperatures were assessed.

Study Design And Methods: Pooled cryoprecipitate was thawed and aliquoted for storage at 1-6°C or 21-24°C. Read More

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http://dx.doi.org/10.1111/trf.15180DOI Listing
April 2019
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Changes in laboratory markers of thrombotic risk early in the first trimester of pregnancy may be linked to an increase in estradiol and progesterone.

Thromb Res 2019 Mar 25;178:47-53. Epub 2019 Mar 25.

Institute of Cardiovascular and Medical Sciences, University of Glasgow, Glasgow, UK.

Background: Pregnant women are at increased risk of venous thrombosis compared to non-pregnant women. Epidemiological and laboratory data suggest that hypercoagulability begins in the first trimester but it is unknown exactly how early in pregnancy this develops. The mechanisms that result in a prothrombotic state may involve oestrogens and progestogens. Read More

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http://dx.doi.org/10.1016/j.thromres.2019.03.015DOI Listing
March 2019
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Effect of six-month use of oral contraceptive pills on plasminogen activator inhibitor-1 & factor VIII among women with polycystic ovary syndrome: An observational pilot study.

Indian J Med Res 2018 Dec;148(Supplement):S151-S155

Department of Clinical Biochemistry/Biochemistry, University of Kashmir, Srinagar, India.

Background & Objectives: Polycystic ovary syndrome (PCOS) is an endocrinopathy warranting lifelong individualized management by lifestyle and pharmacological agents mainly oral contraceptive pills (OCPs). This study was aimed to report the impact of six-month OCP use on plasminogen activator inhibitor-1 (PAI-1) and factor VIII (FVIII) in women with PCOS.

Methods: PCOS women diagnosed on the basis of Rotterdam 2003 criteria, either treated with OCPs (ethinyl estradiol-0. Read More

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http://dx.doi.org/10.4103/ijmr.IJMR_1899_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469381PMC
December 2018
3 Reads

Increased branching and sialylation of N-linked glycans correlate with an improved pharmacokinetic profile for BAY 81-8973 compared with other full-length rFVIII products.

Drug Des Devel Ther 2019 22;13:941-948. Epub 2019 Mar 22.

Biological Development, Bayer US LLC Pharmaceuticals, Berkeley, CA, USA,

Background: BAY 81-8973 (Kovaltry) is an unmodified full-length recombinant factor VIII (rFVIII) for treatment of hemophilia A. The BAY 81-8973 manufacturing process results in a product of enhanced purity with a consistently high degree of branching and sialylation of N-linked glycans. This study evaluated whether a relationship exists between N-linked glycosylation patterns of BAY 81-8973 and two other rFVIII (sucrose-formulated rFVIII [rFVIII-FS; Kogenate FS]) and antihemophilic factor (recombinant) plasma/albumin-free method (rAHF-PFM; Advate) and their pharmacokinetic (PK) characteristics. Read More

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http://dx.doi.org/10.2147/DDDT.S188171DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6435118PMC
March 2019
1 Read

Dental management of patients with haemophilia in the era of recombinant treatments: increased efficacy and decreased clinical risk.

BMJ Case Rep 2019 Apr 8;12(4). Epub 2019 Apr 8.

Universidad Complutense de Madrid, Madrid, Spain.

Haemophilia is a hereditary X-linked recessive disorder caused by a deficiency of either clotting factor VIII (haemophilia A) or IX (haemophilia B). Conventional treatment is currently based on the use of either plasma derived or recombinant coagulation factors. This paper reports on the case of a patient with severe haemophilia who presented with mesial decay and interproximal tartar build-up, for which extraction and scaling to remove tartar deposits were indicated. Read More

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http://dx.doi.org/10.1136/bcr-2018-227974DOI Listing
April 2019
1 Read

Laboratory evidence for hypercoagulability in cirrhotic patients with history of variceal bleeding.

Thromb Res 2019 Apr 1;178:41-46. Epub 2019 Apr 1.

Department of Gastroenterology and Internal Medicine, Medical University of Bialystok, M. Sklodowskiej - Curie 24a, 15-276 Bialystok, Poland.

Aim: We aimed to assess the relationship between procoagulant imbalance and the occurrence of variceal bleeding in patients with liver cirrhosis.

Methods: We compared the results of chromogenic assay for the functional evaluation of the Protein C anticoagulant pathway (ThromboPath®), thromboelastometry and the levels of factor VII, VIII, and antithrombin in two groups of cirrhotic patients: Group 1 (n = 25) - patients with moderate or large esophageal or gastric varices, who had never experienced acute gastrointestinal bleeding and Group 2 (n = 24) - patients with a history of variceal bleeding.

Results: Despite the differences in MELD score and the results of basic laboratory tests indicating more severe cirrhosis and suggesting a greater risk of bleeding in Group 2, the results of thromboelastometry did not differ significantly between groups. Read More

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http://dx.doi.org/10.1016/j.thromres.2019.03.021DOI Listing
April 2019
3 Reads

Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients.

Blood Coagul Fibrinolysis 2019 Apr;30(3):127-132

Hospital General Universitario, Alicante, Spain.

: We hypothesized that inhibitor specificity may predict the outcome of antifactor VIII autoantibodies eradication treatment in acquired hemophilia A. Our objective was to analyze the association between factor VIII domains recognized by inhibitors and outcome of the immunosuppressive therapies (ISTs) in a prospective, observational study. 16 patients were recruited. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000804DOI Listing
April 2019
2 Reads

Reasons for Differences in the Incidence of Venous Thromboembolism in Black Versus White Americans.

Am J Med 2019 Apr 3. Epub 2019 Apr 3.

Department of Medicine, University of Vermont, Colchester Research Facility, 360 South Park Dr., 206D, Colchester, VT 05446, USA; Department of Pathology, University of Vermont, 111 Colchester Avenue, Main Campus, Main Pavilion, Level 2, Burlington, VT 05401, USA. Electronic address:

Introduction: Venous thromboembolism incidence rates are 30-100% higher in American blacks than whites. We examined (a) the degree to which differences in the frequencies of socioeconomic, lifestyle, and medical risk factors, and genetic variants explain the excess venous thromboembolism risk in blacks and (b) whether some risk factors are more strongly associated with venous thromboembolism in blacks compared with whites.

Methods: We measured venous thromboembolism risk factors in black or white participants of the Atherosclerosis Risk in Communities study in 1987-89 and followed them prospectively through 2015 for venous thromboembolism incidence. Read More

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http://dx.doi.org/10.1016/j.amjmed.2019.03.021DOI Listing
April 2019
2 Reads

An update on the 'danger theory' in inhibitor development in haemophilia A.

Expert Rev Hematol 2019 Apr 5. Epub 2019 Apr 5.

a Van Creveldkliniek, University Medical Center Utrecht, Utrecht University , Utrecht , The Netherlands.

Introduction: Nowadays, one of the most serious treatment complications in haemophilia A is the formation of neutralizing antibodies against coagulation factor VIII (FVIII). These so-called inhibitors develop in about 30% of all patients with severe haemophilia A. Once formed, inhibitors reduce FVIII efficacy in blood coagulation, which has a negative impact on patients' health and quality of life and significantly increases haemophilia A treatment costs. Read More

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http://dx.doi.org/10.1080/17474086.2019.1604213DOI Listing
April 2019
2 Reads

[Identification of compound heterozygous mutations of F11 gene in a pedigree affected with heriditary coagulation factor XI deficiency].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2019 Apr;36(4):363-367

Center of Laboratory Medicine, the First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325015, China. Email:

Objective: To identify potential mutations of F11 gene in a pedigree affected with hereditary coagulation factor XI (FXI) deficiency and explore its molecular pathogenesis.

Methods: Prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB), coagulation factor VIII activity (FVIIIC), coagulation factor IX activity (FIXC), coagulation factor XI activity (FXIC), coagulation factor XII activity (FXIIC) and lupus anticoagulation (LA) of the proband and eight family members were determined. FXI antigen (FXIAg) was determined by enzyme-linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2019.04.018DOI Listing
April 2019
5 Reads

Neofunctionalisation of basic helix loop helix proteins occurred when embryophytes colonised the land.

New Phytol 2019 Apr 4. Epub 2019 Apr 4.

Department of Plant Sciences, University of Oxford, South Parks Road, Oxford, OX1 3RB, UK.

•ROOT HAIR DEFECTIVE SIX-LIKE (RSL) genes control the development of structures from single cells at the surface of embryophytes (land plants) such as rhizoids and root hairs. RSL proteins constitute a subclass (VIIIc) of the basic-helix-loop-helix (bHLH) class VIII transcription factor family. •The Charophyceae form the only class of streptophyte algae with tissue-like structures and rhizoids. Read More

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http://dx.doi.org/10.1111/nph.15829DOI Listing
April 2019
3 Reads

Cytokine clearance with CytoSorb® during cardiac surgery: a pilot randomized controlled trial.

Crit Care 2019 Apr 3;23(1):108. Epub 2019 Apr 3.

Adult Intensive Care Unit, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.

Background: Cardiopulmonary bypass (CPB) is often associated with degrees of complex inflammatory response mediated by various cytokines. This response can, in severe cases, lead to systemic hypotension and organ dysfunction. Cytokine removal might therefore improve outcomes of patients undergoing cardiac surgery. Read More

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https://ccforum.biomedcentral.com/articles/10.1186/s13054-01
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http://dx.doi.org/10.1186/s13054-019-2399-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448322PMC
April 2019
11 Reads

Ocular bleeding in an undiagnosed hemophiliac neonate causing irreversible loss of vision: A case report with review of the literature.

Eur J Ophthalmol 2019 Apr 2:1120672119841188. Epub 2019 Apr 2.

1 Department of Ophthalmology, King Abdul-Aziz University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Introduction:: Suprachoroidal hemorrhage is an unexpected complication after penetrating keratoplasty during childhood. We report the case of delayed suprachoroidal hemorrhage after penetrating keratoplasty in a neonate as the first manifestation of hemophilia.

Case Report:: A 4-day-old neonate was referred to our hospital because of bilateral corneal opacities present since birth. Read More

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http://journals.sagepub.com/doi/10.1177/1120672119841188
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http://dx.doi.org/10.1177/1120672119841188DOI Listing
April 2019
3 Reads

How Full-Length FVIII Benefits from Its Heterogeneity - Insights into the Role of the B-Domain.

Pharm Res 2019 Apr 1;36(5):77. Epub 2019 Apr 1.

Research & Development, Baxalta Innovations GmbH, a Takeda company, Vienna, Austria.

Purpose: To explore how the natural heterogeneity of human coagulation factor VIII (FVIII) and the processing of its B-domain specifically modulate protein aggregation.

Methods: Recombinant FVIII (rFVIII) molecular species containing 70% or 20% B-domain, and B-domain-deleted rFVIII (BDD-rFVIII), were separated from full-length recombinant FVIII (FL-rFVIII). Purified human plasma-derived FVIII (pdFVIII) was used as a comparator. Read More

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http://dx.doi.org/10.1007/s11095-019-2599-2DOI Listing

Report of a Rare Case of Epitheliod Hemangioendothelioma in Tongue.

J Dent (Shiraz) 2019 Mar;20(1):70-74

Dept. of Oral and Maxillofacial Pathology, NIMS Dental College, Jaipur, India.

Epitheloid hemangioendothelioma (EHE) is a rare vascular neoplasm of intermediate malignant potential. EHE commonly involves lungs, liver soft tissue, and bone. EHE is extremely rare in tongue and up to our best knowledge only nine cases of EHE of tongue reported in the literature. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421319PMC
March 2019
1 Read

A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia.

Case Rep Hematol 2019 27;2019:8612031. Epub 2019 Feb 27.

Department of Hematology and Oncology, Kita-Harima Medical Center, Hyogo, Japan.

A 67-year-old male, with a known diagnosis of myelodysplastic syndromes with multilineage dysplasia (MDS-MLD) was admitted to our hospital with a primary complaint of subcutaneous bleeding in his left thigh. Laboratory data showed anaemia and prolongation of activated partial thromboplastin time (85.8 s, normal range 24-39 s) without thrombocytopenia. Read More

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http://dx.doi.org/10.1155/2019/8612031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6415290PMC
February 2019
4 Reads

Importance of the Force Field Choice in Capturing Functionally Relevant Dynamics in the von Willebrand Factor.

J Phys Chem Lett 2019 Apr 8;10(8):1928-1934. Epub 2019 Apr 8.

Department of Chemistry, Faculty of Maths & Physical Sciences , University College London , London WC1H 0AJ , United Kingdom.

Whether recent updates and new releases of atomistic force fields can model the structural and dynamical properties of proteins containing both folded and partially disordered domains is still unclear. To address this fundamental question, we tested eight recently released force fields against our set of nuclear magnetic resonance (NMR) observables for a complex and medically relevant system, the major factor VIII binding region on the von Willebrand factor. This biomedically important region comprises both a folded and a partially structured domain. Read More

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http://dx.doi.org/10.1021/acs.jpclett.9b00517DOI Listing
April 2019
1 Read

Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases.

Reumatismo 2019 Apr 1;71(1):37-41. Epub 2019 Apr 1.

Department of Internal Medicine, Pordenone General Hospital.

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Read More

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https://www.reumatismo.org/index.php/reuma/article/view/1041
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http://dx.doi.org/10.4081/reumatismo.2019.1041DOI Listing
April 2019
6 Reads

Risks and patient outcomes of surgical intervention for hemophilic arthropathy.

Expert Rev Hematol 2019 Mar 30:1-9. Epub 2019 Mar 30.

a Department of Orthopedic Surgery , La Paz University Hospital , Madrid , Spain.

Introduction: Surgical interventions usually needed for hemophilic arthropathy include arthroscopic synovectomy, alignment osteotomy, arthroscopic joint debridement, radial head removal, arthrodesis of the ankle, and total joint arthroplasty (elbow, hip, knee, ankle). Areas covered: Whatever the surgical technique, appropriate surgical hemostasis must be achieved through infusion of concentrate of the deficient factor (factor VIII or factor IX), either in recombinant or plasma-derived form, at the correct dosage (ideally for 10-14 days). In patients with inhibitors, there are also the aPCCs (activated prothrombin complex concentrates) and rFVIIa (recombinant activated FVII). Read More

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http://dx.doi.org/10.1080/17474086.2019.1602035DOI Listing
March 2019
2 Reads

Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan.

Diabetes Ther 2019 Mar 29. Epub 2019 Mar 29.

Department of Hematology and Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Chuo-Ku, Fukuoka, 810-8563, Japan.

Herein we describe four patients with acquired hemophilia A (AHA) caused by factor VIII (FVIII) inhibitor and histories of dipeptidyl peptidase-4 inhibitor (DPP4-I) treatment for diabetes mellitus (DM). Drug exposure can cause a breakdown of immune tolerance to FVIII associated with CD4 T cells, resulting in the induction of autoantibodies against FVIII. In patient 1 in the present series, FVIII inhibitor disappeared after DPP4-I treatment. Read More

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http://dx.doi.org/10.1007/s13300-019-0609-3DOI Listing
March 2019
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Quality by Design characterization of the perfusion culture process for recombinant FVIII.

Biologicals 2019 Mar 26. Epub 2019 Mar 26.

Department of Biotechnology, College of Life Sciences and Biotechnology, Korea University, 145, Anam-ro, Seongbuk-gu, Seoul, 02841, Republic of Korea. Electronic address:

A Quality by Design (QbD) concept was applied to characterize a cell culture process for production of the recombinant Factor VIII (rFVIII). We characterized the production bioreactor process and defined the design space by applying risk assessment to determine potential critical process parameters (CPPs) impacting critical quality attributes (CQAs). Characterization studies were subsequently performed using a qualified scaled-down model (SDM) and a multi-factorial design of experiment (DOE) approach to determine both the individual and combined impacts of the potential CPPs on CQAs. Read More

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http://dx.doi.org/10.1016/j.biologicals.2019.03.004DOI Listing
March 2019
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1.209 Impact Factor

Population-based surveillance of haemophilia and patient outcomes in Indiana using multiple data sources.

Haemophilia 2019 Mar 29. Epub 2019 Mar 29.

Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana.

Introduction: Epidemiological surveillance of haemophilia through linkage of medical records within a US state has not been conducted in 20 years.

Aim: The Indiana Haemophilia Surveillance Project aims to identify all persons with haemophilia who resided in Indiana in 2011-2013 and to determine the percentage of patients in Indiana cared for at a federally recognized haemophilia treatment centre (HTC).

Methods: A retrospective review of medical charts was conducted to identify haemophilia cases during the surveillance years. Read More

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http://dx.doi.org/10.1111/hae.13734DOI Listing
March 2019
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Impact of capacity-limited binding on recombinant factor VIII and von Willebrand factor pharmacokinetics in haemophilia A rats.

J Thromb Haemost 2019 Mar 29. Epub 2019 Mar 29.

Haemophilia Research, Global Drug Discovery, Novo Nordisk A/S, Maaloev, Denmark.

Background: Understanding of the pharmacokinetic (PK) interplay between factor VIII (FVIII) and von Willebrand factor (VWF) following high-dose FVIII treatment is lacking.

Objectives: To characterize the PK of recombinant FVIII (rFVIII), VWF and the rFVIII:VWF complex in haemophilia A rats following intravenous administration of rFVIII using PK modeling. A second aim was to investigate the impact of high daily dosing and constant expression of rFVIII on VWF exposure using PK simulations. Read More

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http://dx.doi.org/10.1111/jth.14441DOI Listing
March 2019
3 Reads

Factor VIII: Long-established role in haemophilia A and emerging evidence beyond haemostasis.

Blood Rev 2019 05 3;35:43-50. Epub 2019 Mar 3.

Georgetown University Medical Center, Washington, DC, USA.

Factor VIII protein (FVIII) as a coagulation replacement factor has for decades been used as the standard of care for management of people with haemophilia A. It is effective for treatment of bleeding events, as prophylaxis to prevent bleeding events and preserve joint function, and to support surgery in people with haemophilia A. Despite long experience in treating haemophilia A, we are only beginning to understand the functions of FVIII beyond its established role as a coenzyme to factor IXa to expedite thrombin generation through the intrinsic pathway of coagulation. Read More

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http://dx.doi.org/10.1016/j.blre.2019.03.002DOI Listing
May 2019
2 Reads

[PREGNANCY RELATED ACQUIRED HEMOPHILIA A].

Harefuah 2019 Mar;158(3):184-186

Hematology Institute and Blood Bank.

Introduction: Acquired hemophilia A is a rare disease. The incidence has been estimated to be 1.3-1. Read More

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March 2019
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[HEMOPHILIA - A ROYAL DISEASE IN THE HOLY LAND].

Harefuah 2019 Mar;158(3):173-175

National Hemophilia Center and Thrombosis Institute, Sheba Medical Center, Tel Hashomer, Israel.

Introduction: Hemophilia is a hereditary congenital hemorrhagic diathesis caused by mutations in blood coagulation factor VIII (FVIII) or IX (FIX) genes, causing hemophilia A and B, respectively. Most cases are familial but a significant minority is sporadic.

Objectives: To examine the presenting symptoms of patients with hemophilia in Israel and identify causes for delay in diagnosis. Read More

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March 2019
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[ACQUIRED HEMOPHILIA A AND THE TIMING OF IMMUNOMODULATORY THERAPY].

Harefuah 2019 Mar;158(3):165-167

Thrombosis and Hemostasis Unit, Rambam Health Care Campus, Haifa, Israel.

Introduction: Acquired hemophilia A is an autoimmune disease affecting men and women equally and is idiopathic in 50% of the cases. As the mortality rate reaches 50%, prompt diagnosis and treatment are needed. Diagnosis is made in a patient with a bleeding manifestation and prolonged PTT (partial thromboplastin time) that is not corrected in a mixing study with normal plasma. Read More

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March 2019
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Sambucus australis Modulates Inflammatory Response via Inhibition of Nuclear Factor Kappa B (NF-kB) in vitro.

An Acad Bras Cienc 2019 Mar 21;91(1):e20170831. Epub 2019 Mar 21.

Laboratório de Imunofarmacologia e Biologia Molecular, Instituto de Ciências da Saúde, Universidade Federal da Bahia, Avenida Reitor Calmon, s/n, Vale do Canela, 41110-100 Salvador, BA, Brazil.

Medicinal plants have long been used as an alternative to traditional drugs for the treatment of inflammatory conditions due to the classical side effects and restricted access of various commercially available drugs, such as steroids (GCs) and nonsteroidal anti-inflammatory drugs (NSAIDs). Sambucus australis is a Brazilian herb that is commonly used to treat inflammatory diseases; however, few studies have examined the use of this species in the treatment of inflammatory conditions. The present study aims to evaluate the potential anti-inflammatory activity of S. Read More

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http://dx.doi.org/10.1590/0001-3765201920170831DOI Listing
March 2019
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Elevated fibrinogen, von Willebrand factor, and Factor VIII confer resistance to dilutional coagulopathy and activated protein C in normal pregnant women.

Br J Anaesth 2019 Mar 18. Epub 2019 Mar 18.

Department of Anesthesiology, University of Maryland School of Medicine, Baltimore, MD, USA.

Background: Gestational changes in coagulation factor concentrations include elevations in fibrinogen, Factor VIII, and von Willebrand factor (vWF). We hypothesised that blood samples from term pregnant (TP) subjects are less prone to coagulation disturbances from haemodilution compared with those from non-pregnant (NP) females.

Methods: Blood samples were collected from 15 NP and 15 TP subjects. Read More

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http://dx.doi.org/10.1016/j.bja.2019.02.012DOI Listing
March 2019
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Heart Rate in Hypertension: Review and Expert Opinion.

Int J Hypertens 2019 19;2019:2087064. Epub 2019 Feb 19.

Dept. of Cardiology, Sir Ganga Ram Hospital, New Delhi, India.

Heart rate (HR) is strongly associated with both peripheral and central blood pressures. This association has implications in hypertension (HTN) prognosis and management. Elevated HR in HTN further elevates the risk of adverse outcomes. Read More

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http://dx.doi.org/10.1155/2019/2087064DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399539PMC
February 2019
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[Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review].

Rev Med Interne 2019 Mar 20. Epub 2019 Mar 20.

Service d'hématologie, CHU Nîmes, 30029 Nîmes, France.

Introduction: Autoimmune acquired haemophilia is a rare autoimmune disease. The purpose of immunosuppressive therapy is to stop the production of autoantibodies that inhibit clotting factors VIII or IX. A corticosteroids-cyclophosphamide combination is recommanded as first-line therapy. Read More

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http://dx.doi.org/10.1016/j.revmed.2019.02.008DOI Listing
March 2019
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Evaluation of milk fat globule-epidermal growth factor-factor VIII and IL-1β levels in gingival crevicular fluid and saliva in periodontal disease and health.

Odontology 2019 Mar 22. Epub 2019 Mar 22.

Department of Periodontology, Faculty of Dentistry, University of Health Science, Istanbul, Turkey.

The aim of this study is to determine the levels of MFG-E8 and interleukin (IL)-1β in saliva and gingival crevicular fluid (GCF) associated with periodontal health and disease. Whole saliva and GCF samples were obtained from systemically healthy participants who were either periodontally healthy (n = 24) or suffered from gingivitis (n = 25) or chronic periodontitis (n = 25). Full-mouth clinical periodontal measurements, including bleeding on probing, probing depth, gingival index, plaque index, and clinical attachment level were also recorded. Read More

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http://dx.doi.org/10.1007/s10266-019-00419-5DOI Listing
March 2019
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Single-molecule measurement and bioinformatics analysis suggest a preferred orientation of human coagulation factor VIII on hydrophobic interfaces.

Biophys Chem 2019 05 15;248:9-15. Epub 2019 Mar 15.

Zhangjiang Lab, Shanghai Advanced Research Institute, Chinese Academy of Sciences, Shanghai 201210, China; CAS Key Laboratory of Interfacial Physics and Technology, Shanghai Institute of Applied Physics, Chinese Academy of Sciences, Shanghai 201800, China. Electronic address:

Investigating the adsorption behavior of coagulation proteins on interfaces will contribute to better understating blood clotting and to the development of biocompatible materials. In this work, atomic force microscopy (AFM)-based peakforce quantitative nanomechanical mapping (PF-QNM) was combined with bioinformatics tool to study the adsorption and orientation of coagulation factor VIII (FVIII) on both hydrophilic and hydrophobic interfaces by the height and mechanical measurement of single protein molecules. We found that interfacial hydrophilicity/hydrophobicity greatly influence the heights and Young's modulus of individual proteins. Read More

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http://dx.doi.org/10.1016/j.bpc.2019.03.001DOI Listing
May 2019
1 Read
1.986 Impact Factor

Utilizing Plasma Composition Data to Help Determine Procoagulant Dynamics in Patients with Thermal Injury: A Computational Assessment.

Mil Med 2019 Mar;184(Supplement_1):392-399

The Department of Biochemistry, College of Medicine, University of Vermont, 360 South Park Drive, Colchester, VT.

Introduction: The development of methods that generate individualized assessments of the procoagulant potential of burn patients could improve their treatment. Beyond its role as an essential intermediate in the formation of thrombin, factor (F)Xa has systemic effects as an agonist to inflammatory processes. In this study, we use a computational model to study the FXa dynamics underlying tissue factor-initiated thrombin generation in a small cohort of burn patients. Read More

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http://dx.doi.org/10.1093/milmed/usy397DOI Listing
March 2019
3 Reads

Critical Evaluation of Strategies for the Production of Blood Coagulation Factors in Plant-Based Systems.

Front Plant Sci 2019 7;10:261. Epub 2019 Mar 7.

Plant Biotechnology, Faculty of Biology, University of Freiburg, Freiburg im Breisgau, Germany.

The use of plants as production platforms for pharmaceutical proteins has been on the rise for the past two decades. The first marketed plant-made pharmaceutical, taliglucerase alfa against Gaucher's disease produced in carrot cells by Pfizer/Protalix Biotherapeutics, was approved by the US Food and Drug Administration (FDA) in 2012. The advantages of plant systems are low cost and highly scalable biomass production compared to the fermentation systems, safety compared with other expression systems, as plant-based systems do not produce endotoxins, and the ability to perform complex eukaryotic post-translational modifications, e. Read More

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http://dx.doi.org/10.3389/fpls.2019.00261DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6417376PMC
March 2019
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Inflammatory and immune response genes: A genetic analysis of inhibitor development in Iranian hemophilia A patients.

Pediatr Hematol Oncol 2019 Feb 19;36(1):28-39. Epub 2019 Mar 19.

c Department of Hepatitis and AIDS , Pasteur Institute of Iran , Tehran , Iran.

A major problem of hemophilia A (HA) treatment is the development of factor VIII (FVIII) inhibitor, which usually occurs shortly after initiating replacement therapy. Several studies showed the correlation between inhibitor development and polymorphisms in inflammatory and immune response genes of HA patients; however, literature data are not available to prove this association in Iranian population. The aim of this study was to investigate a possible association between FVIII inhibitor formation and the polymorphisms of 16 inflammatory and immune response genes in Iranian severe HA patients (FVIII activity < 1%). Read More

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http://dx.doi.org/10.1080/08880018.2019.1585503DOI Listing
February 2019
1 Read