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Hemophilia A

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https://www.readbyqxmd.com/read/28932824/factor-viia-interaction-with-epcr-modulates-the-hemostatic-effect-of-rfviia-in-hemophilia-therapy-mode-of-its-action
#1
Shiva Keshava, Jagan Sundaram, Anuradha Rajulapati, Charles Esmon, Usha Pendurthi, L Vijaya Mohan Rao
Recent studies established that clotting factor VIIa (FVIIa) binds endothelial cell protein C receptor (EPCR). It has been speculated that FVIIa interaction with EPCR might augment the hemostatic effect of rFVIIa in therapeutic conditions. The present study is carried out to investigate the mechanism by which FVIIa interaction with EPCR contributes to the hemostatic effect of rFVIIa in hemophilia therapy. Active-site inhibited FVIIa, which is capable of binding to EPCR but has no ability to activate factor X, reduced the concentration of rFVIIa required to correct the bleeding following the saphenous vein injury in mouse hemophilia model systems...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/28932649/hepatocyte-transplantation-cell-sheet-technology-for-liver-cell-transplantation
#2
REVIEW
Kohei Tatsumi, Teruo Okano
PURPOSE OF REVIEW: We will review the recent developments of cell sheet technology as a feasible tissue engineering approach. Specifically, we will focus on the technological advancement for engineering functional liver tissue using cell sheet technology, and the associated therapeutic effect of cell sheets for liver diseases, highlighting hemophilia. RECENT FINDINGS: Cell-based therapies using hepatocytes have recently been explored as a new therapeutic modality for patients with many forms of liver disease...
2017: Current Transplantation Reports
https://www.readbyqxmd.com/read/28922951/a-retrospective-study-of-cytokine-profiles-changes-in-mice-with-fviii-inhibitor-development-after-aav-mediated-gene-therapy-in-hemophilia-a-mouse-model
#3
Junjiang Sun, Zhenhua Yuan, Yasmina L Abajas, Dorreen E Szollosi, Genlin Hu, Baolai Hua, Xiao Xiao, Chengwen Li
The development of inhibitory autoantibodies to the infused clotting factor VIII is a major complication for severe hemophilia A management. Novel therapy options for hemophilia have significantly progressed in the last decade and a gene therapy cure for hemophilia is translating into reality. However, mechanistic studies of FVIII autoantibodies (FVIII inhibitors) have lagged behind and remain a challenge for both protein replacement and gene therapy. FVIII inhibitor formation is assumed to be a classical T cell-dependent immune response in which cytokines/chemokines play an important role...
September 19, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28921445/quantification-of-coagulation-factor-viii-by-selective-reaction-monitoring
#4
Edson Galdino do Nascimento Filho, Guilherme Pauperio Lanfredi, Mário Soares Abreu-Neto, Dimas Tadeu Covas, Vitor Marcel Faça
Coagulation factor VIII (FVIII) is an important glycoprotein involved in the extrinsic coagulation cascade. Mutations in FVIII gene results in hemophilia A, a recessive coagulation disorder that is clinically managed by administration of purified FVIII from blood donors or recombinant FVIII. Because of its fundamental therapeutic application, biotechnological production of FVIII requires rigid quality control and monitoring in patients and clinical trials. Here, we describe a protocol for a mass spectrometry based approach termed selective reaction monitoring (SRM) as an important alternative tool for accurate and sensitive quantitation of purified or recombinant FVIII...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28921441/purification-and-autoactivation-method-for-recombinant-coagulation-factor-vii
#5
Vladimir Granovski, Marcela C C Freitas, Mario Soares Abreu-Neto, Dimas T Covas
Recombinant coagulation factor VII is a very important and complex protein employed for treatment of hemophiliac patients (hemophilia A/B) who develop inhibitors antibodies to conventional treatments (FVIII and FIX). The rFVII is a glycosylated molecule and circulates in plasma as zymogen of 50 kDa. When activated the molecule is cleaved to 20-30 kDa and has a half-life of about 3 h, needing to be processed fast and efficiently until freeze-drying. Here, we describe a very simple and fast purification sequence for rFVII using affinity FVII Select resin and a dialysis system that can be easily scaled up...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28920105/the-impact-of-von-willebrand-factor-on-factor-viii-memory-immune-responses
#6
Juan Chen, Jocelyn A Schroeder, Xiaofeng Luo, Qizhen Shi
Immune tolerance induction (ITI) with aggressive infusion of factor VIII (FVIII) is the current strategy used to eradicate FVIII inhibitors and restore normal FVIII pharmacokinetics in inhibitor patients. Whether the use of FVIII products containing von Willebrand factor (VWF) will affect the efficacy of ITI is still controversial. In this study, we explored the impact of VWF on FVIII memory immune responses in hemophilia A (HA) mice. A T-cell proliferation assay and cytokine profile analysis were used to study FVIII-primed CD4(+) T cells...
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/28919830/identification-of-patients-with-congenital-hemophilia-in-a-large-electronic-health-record-database
#7
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) are an important source of information with regard to diagnosis and treatment of rare health conditions, such as congenital hemophilia, a bleeding disorder characterized by deficiency of factor VIII (FVIII) or factor IX (FIX). OBJECTIVE: To identify patients with congenital hemophilia using EHRs. DESIGN: An EHR database study. SETTING: EHRs were accessed from Humedica between January 1, 2007, and July 31, 2013...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28919213/treatment-and-complications-in-acquired-hemophilia-a-experience-from-a-single-center
#8
Laura Entrena Ureña, Dolores Fernández Jiménez, María José Gutiérrez Pimentel
No abstract text is available yet for this article.
September 14, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28912633/a-preliminary-case-series-evaluating-the-safety-and-immediate-to-short-term-clinical-benefits-of-joint-mobilization-in-hemophilic-arthritis-of-the-lower-limb
#9
Emma Scaddan, John Rowell, Shaun O'Leary
OBJECTIVES: Arthritis resulting from recurrent intra-articular bleeding in individuals with hemophilia can be severely debilitating due to joint pain and stiffness with subsequent loss of mobility and function. Very limited studies have investigated the potential benefits of joint mobilization for this condition. This case series is a preliminary investigation of safety, as well as immediate and short-term clinical benefits, associated with gentle knee and ankle joint mobilization in people with hemophilic arthropathy...
September 2017: Journal of Manual & Manipulative Therapy
https://www.readbyqxmd.com/read/28905885/long-term-correction-of-hemophilia-a-mice-following-lentiviral-mediated-delivery-of-an-optimized-canine-factor-viii-gene
#10
J M Staber, M J Pollpeter, C-G Anderson, M Burrascano, A L Cooney, P L Sinn, D T Rutkowski, W C Raschke, P B McCray
Current therapies for hemophilia A include frequent prophylactic or on-demand intravenous factor treatments which are costly, inconvenient and may lead to inhibitor formation. Viral vector delivery of factor VIII (FVIII) cDNA has the potential to alleviate the debilitating clotting defects. Lentiviral-based vectors delivered to murine models of hemophilia A mediate phenotypic correction. However, a limitation of lentiviral-mediated FVIII delivery is inefficient transduction of target cells. Here, we engineer a feline immunodeficiency virus (FIV) -based lentiviral vector pseudotyped with the baculovirus GP64 envelope glycoprotein to mediate efficient gene transfer to mouse hepatocytes...
September 14, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28904709/-factor-v-congenital-deficiency-about-a-case
#11
Saloua Boujrad, Brahim El Hasbaoui, Hanae Echahdi, Mohamed Malih, Aomar Agadr
Factor V congenital deficiency is a rare coagulation disorder initially described by Owren in 1947 and known as para hemophilia. It is transmitted through autosomal-recessive inheritance and homozygous cases are usually symptomatic. Factor V is an essential cofactor in the conversion of prothrombin to thrombin by activated factor X. In the absence of factor V, thrombin generation is slowed down and fibrin formation is delayed. This results in a bleeding tendency. We report a case of factor V congenital deficiency in an infant with recurrent epistaxis...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28903884/successful-outcome-of-severe-intra-cerebral-bleeding-associated-with-acquired-factor-v-inhibition-utilization-of-multiple-therapeutic-agents
#12
Panagiotis Andreadis, Katerina Kafantari, Aleka Agapidou, Sofia Vakalopoulou, Efthymia Vlachaki
BACKGROUND: Acquired coagulation Factor inhibitors are antibodies that either inhibit the activity or increase the clearance of a clotting factor leading to an increased risk of bleeding. Most of the times, the disorder is attributed to Factor VIII inhibition (Acquired Hemophilia A), however other coagulation factors could also be implicated. CASE REPORT: We herein would like to report an interesting case of a patient who underwent Coronary Artery Bypass Graft and received antibiotic treatment after surgery with a third-generation cephalosporin...
September 13, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28901996/intracranial-hemorrhage-in-congenital-bleeding-disorders
#13
Shadi Tabibian, Hoda Motlagh, Majid Naderi, Akbar Dorgalaleh
: Intracranial hemorrhage (ICH), as a life-threatening bleeding among all kinds of congenital bleeding disorders (CBDs), is a rare manifestation except in factor XIII (FXIII) deficiency, which is accompanied by ICH, early in life, in about one-third of patients. Most inherited platelet function disorders (IPFDs) are mild to moderate bleeding disorders that can never experience a severe bleeding as in ICH; however, Glanzmann's thrombasthenia, a common and severe inherited platelet function disorder, can lead to ICH and occasional death...
September 9, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28900823/health-related-quality-of-life-developmental-milestones-and-self-esteem-in-young-adults-with-bleeding-disorders
#14
P F Limperg, L Haverman, H Maurice-Stam, M Coppens, C Valk, M J H A Kruip, J Eikenboom, M Peters, M A Grootenhuis
BACKGROUND: The treatment of bleeding disorders improved in the last decades. However, the effect of growing up with bleeding disorders on developmental, emotional, and social aspects is understudied. Therefore, this study assesses HRQOL, developmental milestones, and self-esteem in Dutch young adults (YA) with bleeding disorders compared to peers. METHODS: Ninety-five YA (18-30 years) with bleeding disorders (78 men; mean 24.7 years, SD 3.5) and 17 women (mean 25...
September 12, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28895852/gene-therapy-for-hemophilia
#15
REVIEW
Amit C Nathwani, Andrew M Davidoff, Edward G D Tuddenham
The best currently available treatments for hemophilia A and B (factor VIII or factor IX deficiency, respectively) require frequent intravenous infusion of highly expensive proteins that have short half-lives. Factor levels follow a saw-tooth pattern that is seldom in the normal range and falls so low that breakthrough bleeding occurs. Most hemophiliacs worldwide do not have access to even this level of care. In stark contrast, gene therapy holds out the hope of a cure by inducing continuous endogenous expression of factor VIII or factor IX following transfer of a functional gene to replace the hemophilic patient's own defective gene...
October 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28895303/coronary-angiography-with-or-without-percutaneous-coronary-intervention-in-patients-with-hemophilia-systematic-review
#16
Christian Boehnel, Hans Rickli, Lukas Graf, Micha T Maeder
OBJECTIVES: We aimed to summarize the evidence for periprocedural and long-term strategies to both minimize the bleeding risk and ensure sufficient anticoagulation and antiaggregation in hemophilia patients undergoing coronary angiography with or without percutaneous coronary interventions (PCI). BACKGROUND: Hemophilia patients undergoing coronary angiography and PCI are at risk of bleeding due to deficiency of the essential clotting factors VIII or IX combined with the need of peri-interventional anticoagulation and antiaggregation and dual antiplatelet therapy (DAPT) after PCI...
September 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28891453/management-of-hemophilic-arthropathy-of-the-ankle
#17
E Carlos Rodriguez-Merchan
BACKGROUND: Hemophilic arthropathy (HA) of the ankle is prevalent in people with hemophilia (PWH). It is frequently severe and incapacitating, due to recurrent bleeding into the ankle articulation during infancy. Around 50% of hemophilic patients suffer from ankle pain and radiological signs of HA. OBJECTIVE: To review current treatment of HA of the ankle in PWH. METHOD: A literature review of hemophilic ankle arthropathy in PWH was performed utilizing MEDLINE (PubMed) and the Cochrane Library...
September 7, 2017: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/28886353/initial-manifestation-of-acquired-hemophilia-a-after-a-routine-tooth-extraction-a-case-report-and-literature-review
#18
Nicholas A Bennetts, James E Mergelmeyer, Eric J Reimer, James C Melville
Although surgical treatment of patients on anticoagulation regimens is common practice among oral and maxillofacial surgeons, unexpected and unknown coagulopathies can have devastating and catastrophic consequences for the most routine of procedures. Acquired hemophilia A (AHA) is an extremely rare life-threatening bleeding disorder characterized by autoantibodies directed against circulating coagulation factor VIII. The effects of AHA can produce catastrophic bleeding and hematomas. The effect of this uncontrolled hemorrhage after dentoalveolar surgery can mimic severe head and neck infection by causing dysphagia, odynophagia, and acute airway complications...
August 12, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28884611/an-evaluation-of-the-activated-partial-thromboplastin-time-waveform
#19
Takeshi Matsumoto, Hideo Wada, Naoki Fujimoto, Junki Toyoda, Yasunori Abe, Kohshi Ohishi, Yoshiki Yamashita, Makoto Ikejiri, Kei Hasegawa, Kei Suzuki, Hiroshi Imai, Kaname Nakatani, Naoyuki Katayama
The activated partial thromboplastin time (APTT) waveform includes several parameters that are related to various underlying diseases. The APTT waveform was examined in various diseases. Regarding the pattern of APTT waveform, a biphasic pattern of the first or second derivative curve (DC) was observed in patients with hemophilia and patients positive for antiphospholipid (aPL) antibodies or coagulation factor VIII (FVIII) inhibitors. The time of the first and second DC and fibrin formation at 1/2 height were prolonged in patients with hemophilia, patients with inhibitors, patients positive for aPL, patients treated with anti-Xa agents, and patients with disseminated intravascular coagulation (DIC)...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28883743/medical-decision-making-and-risky-choices-psychological-and-medicolegal-consequences-of-hiv-and-hcv-contamination-of-blood-products
#20
S Riva, S Del Sordo, U Genovese, G Pravettoni
AIMS: The overall goal of this article is to make a scientific comment about the psycho-social consequences of hemophilia patients affected by human immunodeficiency virus (HIV) and/or hepatitis C virus (HCV) and to point out the related medicolegal issues. METHODS: This commentary takes into account some published evidences about the current scenario of hemophilia patients infected by HIV and/or HCV who received contaminated blood products in the late 1970s through 1985...
2017: HIV/AIDS: Research and Palliative Care
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