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hemophilia review

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https://www.readbyqxmd.com/read/28103444/efficacy-and-safety-of-a-recombinant-factor-viii-produced-from-a-human-cell-line-simoctocog-alfa
#1
Massimo Franchini, Pier Mannuccio Mannucci
The development of inhibitors against infused factor VIII (FVIII) has a detrimental impact on health and quality of life of patients with hemophilia A. Several observational studies and a recently published randomized trial indicate that the inhibitor risk in previously untreated patients (PUPs) is higher following the use of recombinant FVIII (rFVIII) products compared with plasma-derived FVIII concentrates. There is currently a great interest towards newer rFVIII products that adopt various technological solutions to reduce the inhibitor risk...
January 19, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28044933/should-arterial-embolization-in-recurrent-spontaneous-hemophilic-hemarthroses-refractory-to-intensive-prophylaxis-be-the-first-invasive-resort
#2
E Carlos Rodriguez-Merchan
This paper reviews the role of arterial embolization (AE) in recurrent spontaneous hemarthroses (RSH) that are refractory to intensive prophylaxis (RIP) in people with hemophilia (PWH). There are six articles in the literature on the topic. The total number of AEs performed so far is 78 in 69 patients. Four second AEs were required (4/78), and a third AE in one (1/78). No serious adverse effects have been reported except for a pseudoaneurym (1/78) of the femoral artery at the puncture site (that eventually required surgical repair) and for one patient (1/78) that had recurrence of bleeding for whom surgical exploration was required...
December 16, 2016: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/28036020/bispecific-antibodies-as-a-development-platform-for-new-concepts-and-treatment-strategies
#3
REVIEW
Fa Yang, Weihong Wen, Weijun Qin
With the development of molecular cloning technology and the deep understanding of antibody engineering, there are diverse bispecific antibody formats from which to choose to pursue the optimal biological activity and clinical purpose. The single-chain-based bispecific antibodies usually bridge tumor cells with immune cells and form an immunological synapse because of their relatively small size. Bispecific antibodies in the IgG format include asymmetric bispecific antibodies and homodimerized bispecific antibodies, all of which have an extended blood half-life and their own crystalline fragment (Fc)-mediated functions...
December 28, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28030484/pharmacokinetics-safety-and-efficacy-of-recombinant-factor-viii-fc-fusion-protein-a-practical-review
#4
Kim Schafer, James Munn, Kate Khair, Neelam Thukral, Angela Tom, Sally McAlister
Prophylaxis for hemophilia A with conventional factor VIII (FVIII) products requires frequent intravenous dosing, which may reduce adherence. Recombinant factor VIII Fc fusion protein (rFVIIIFc) has a prolonged half-life compared with conventional rFVIII, and has demonstrated safety and efficacy for the prevention and treatment of bleeding episodes in phase 3 studies of patients with severe hemophilia A. Most subjects experienced reduced prophylactic dosing frequency with rFVIIIFc compared with prestudy FVIII; the median total weekly prophylactic consumption was comparable...
January 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28025929/hemophilia-management-via-data-collection-and-reporting-initial-findings-from-the-comprehensive-care-sustainability-collaborative
#5
Michael D Tarantino, Vanita K Pindolia
BACKGROUND: Despite being a rare disorder, hemophilia represents a significant driver of health care resource utilization and requires expert hematologic and multidisciplinary services to achieve optimal outcomes. Since their inception nearly 40 years ago, hemophilia treatment centers (HTCs) have served as centers of excellence in providing the intensive care and ancillary services necessary for this unique patient base; however, the full capabilities of these centers may be underused in the current framework of managed care, largely because of a lack of communication and information shared between payers and HTC stakeholders...
January 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/27995035/clinical-and-imaging-analysis-of-subclinical-hemophilia-combined-with-coxarthrosis-case-report-and-literature-review
#6
Cheng Liu, Jun Guo, Qiu Cui, Dingfeng Li, Yanjun Zeng
INTRODUCTION: The incidence of subclinical hemophilic arthritis is low, with this case reports and literature review, we hope clinicians could pay more attention to the diagnosis of subclinical hemophilic arthritis and prevent the misdiagnosis and mistreatment. CASE PRESENTATION: We analyzed the imaging feature, and therapy of a subclinical hemophilia case with hip lesion by reporting its clinical manifestations, imaging features and therapy procedure, and reviewing literatures...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27992070/exercise-for-haemophilia
#7
REVIEW
Karen Strike, Kathy Mulder, Rojer Michael
BACKGROUND: Haemophilia is a bleeding disorder associated with haemorrhaging into joints and muscles. Exercise is often used to aid recovery after bleeds, and to improve joint function in the presence of arthropathy. OBJECTIVES: Our objective was to systematically review the available evidence on the safety and effectiveness of exercise for people with haemophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register and electronic databases PubMed, OVID-Embase, and CINAHL...
19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27977390/development-of-a-web-accessible-population-pharmacokinetic-service-hemophilia-wapps-hemo-study-protocol
#8
Alfonso Iorio, Arun Keepanasseril, Gary Foster, Tamara Navarro-Ruan, Alanna McEneny-King, Andrea N Edginton, Lehana Thabane
BACKGROUND: Individual pharmacokinetic assessment is a critical component of tailored prophylaxis for hemophilia patients. Population pharmacokinetics allows using individual sparse data, thus simplifying individual pharmacokinetic studies. Implementing population pharmacokinetics capacity for the hemophilia community is beyond individual reach and requires a system effort. OBJECTIVE: The Web-Accessible Population Pharmacokinetic Service-Hemophilia (WAPPS-Hemo) project aims to assemble a database of patient pharmacokinetic data for all existing factor concentrates, develop and validate population pharmacokinetics models, and integrate these models within a Web-based calculator for individualized pharmacokinetic estimation in patients at participating treatment centers...
December 15, 2016: JMIR Research Protocols
https://www.readbyqxmd.com/read/27918159/-pregnancy-in-women-with-congenital-bleeding-disorder
#9
T Binder, P Salaj, V Komrska
THE AIM OF THE STUDY: To highlight the risks associated with pregnancy at women with von Willebrand´s disease or hemophilia. Introduce the rules of multidisciplinary prenatal and peripartal care to minimalize these risks. The article is accompanied by case report where maladministration led to fatal consequences for the newborn. DESIGN: Review and case report.Seatings: Department Obstetric and Gynecology UJEP and Masaryk´s Hospital Ústí n/Labem, Institute Haematology and Blood Transfer Prague, Children´s Haemato-onkology Clinic University Hospital Prague Motol...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27913543/hemophilia-and-inhibitors-current-treatment-options-and-potential-new-therapeutic-approaches
#10
Shannon L Meeks, Glaivy Batsuli
The immune response to infused factor concentrates remains a major source of morbidity and mortality in the treatment of patients with hemophilia A and B. This review focuses on current treatment options and novel therapies currently in clinical trials. After a brief review of immune tolerance regimens, the focus of the discussion is on preventing bleeding in patients with hemophilia and inhibitors. Recombinant factor VIIa and activated prothrombin complex concentrates are the mainstays in treating bleeds in patients with inhibitors...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27906086/massive-adrenal-vein-aneurysm-mimicking-an-adrenal-tumor-in-a-patient-with-hemophilia-a-a-case-report-and-review-of-the-literature
#11
Richard Sleightholm, Steven Wahlmeier, Jeffrey S Carson, Andjela Drincic, Audrey Lazenby, Jason M Foster
BACKGROUND: Visceral venous aneurysms are exceedingly rare, and until now, there have been no reports of this phenomenon in the adrenal vasculature. This report details the first adrenal venous aneurysm reported in the literature. The aneurysm presented as an 18-cm mass that was initially suspected to be a hematoma or tumor on the basis of the complex medical history of the patient, which included hemophilia A and testicular cancer. After surgical excision, pathologic examination confirmed this mass to be a 15...
December 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27897038/adeno-associated-virus-gene-therapy-for-liver-disease
#12
Lisa M Kattenhorn, Christopher H Tipper, Lorelei Stoica, Deborah S Geraghty, Teresa L Wright, K Reed Clark, Samuel C Wadsworth
The field of adeno-associated virus (AAV) gene therapy has progressed rapidly over the past decade, with the advent of novel capsid serotype and organ-specific promoters, and an increasing understanding of the immune response to AAV administration. In particular, liver-directed therapy has made remarkable strides, with a number of clinical trials currently planned and ongoing in hemophilia A and B, as well as other liver disorders. This review focuses on liver-directed AAV gene therapy, including historic context, current challenges, and future developments...
December 2016: Human Gene Therapy
https://www.readbyqxmd.com/read/27890816/pathophysiology-of-hemophilic-arthropathy-and-potential-targets-for-therapy
#13
REVIEW
Astrid E Pulles, Simon C Mastbergen, Roger E G Schutgens, Floris P J G Lafeber, Lize F D van Vulpen
Hemophilia is a congenital clotting factor deficiency characterized by spontaneous and trauma-related bleeding. Spontaneous bleeding shows a predilection for joints, and repeated hemarthroses lead to a disabling condition called hemophilic arthropathy. Treatment of this condition consists of preventing joint bleeding on the one hand and orthopedic surgery as a last resort on the other. Up till now, there is no disease modifying therapy available to fill the gap between these extremes. This review provides an overview of the pathogenesis of hemophilic arthropathy in order to identify potential targets for therapy...
January 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/27885871/continuous-prophylaxis-with-recombinant-factor-ix-fc-fusion-protein-and-conventional-recombinant-factor-ix-products-comparisons-of-efficacy-and-weekly-factor-consumption
#14
Alfonso Iorio, Sangeeta Krishnan, Karl-Johan Myrén, Stefan Lethagen, Nora McCormick, Sander Yermakov, Paul Karner
BACKGROUND: Continuous prophylaxis for patients with hemophilia B requires frequent injections that are burdensome and that may lead to suboptimal adherence and outcomes. Hence, therapies requiring less-frequent injections are needed. In the absence of head-to-head comparisons, this study compared the first extended-half-life-recombinant factor IX (rFIX) product-recombinant factor IX Fc fusion protein (rFIXFc)-with conventional rFIX products based on annualized bleed rates (ABRs) and factor consumption reported in studies of continuous prophylaxis...
December 21, 2016: Journal of Medical Economics
https://www.readbyqxmd.com/read/27882429/endovascular-management-of-recurrent-spontaneous-hemarthrosis-after-arthroplasty
#15
Marcin K Kolber, Pratik A Shukla, Abhishek Kumar, Adam Zybulewski, Todd Markowitz, James E Silberzweig
PURPOSE: Recurrent spontaneous hemarthrosis is an infrequent but debilitating late complication of joint replacement, affecting up to 1.6% of patients with arthroplasty of the affected joint. Repeated episodes of bleeding result in an inflammatory cascade that further propagates bleeding events. Open and arthroscopic synovectomy are often performed when conservative treatments fail. Transarterial embolization is increasingly utilized as a less invasive option; however, its role is not widely established...
November 23, 2016: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/27868097/percutaneous-nephrolithotomy-in-rare-bleeding-disorders-a-case-report-and-review-of-the-literature
#16
Ali Ersin Zumrutbas, Cihan Toktas, Aykut Baser, Omer Levent Tuncay
Surgery in patients with congenital or acquired coagulation defects has always been challenging and requires special care with a multidisciplinary approach. Percutaneous nephrolithotomy (PCNL) is a standard procedure performed in patients with kidney stones. Although prone to bleeding more than most of the widely performed surgical procedures, there are not much data regarding PCNL in patients with bleeding disorders or coagulation defects. There are only case reports or series with a small number of patients for the patients with common coagulation defects, including hemophilias...
2016: Journal of Endourology Case Reports
https://www.readbyqxmd.com/read/27833501/anesthetic-management-of-patient-with-hemophilia-a-undergoing-emergency-ventriculoperitoneal-shunting-a-case-report-and-review-of-literature
#17
Rashid Saeed Khokhar, Altaf Hussain, Mueen Ullah Khan, Muhammad Hajnour, Sadia Qureshi, Mansoor Aqil
Hemophilia A is a hemorrhagic trend almost exclusively affecting males (X-related recessive disease). In 85% of cases, it is caused by factor VIII deficiency, called hemophilia A or classic hemophilia. Successful anesthetic management depends on the special care and a multidisciplinary team of health professionals informed about the disease, including qualified hematologist, surgeon, and anesthesiologist.
October 2016: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/27818588/update-on-occult-hepatitis-b-virus-infection
#18
REVIEW
Manoochehr Makvandi
The event of mutations in the surface antigen gene of hepatitis B virus (HBV) results in undetectable hepatitis B surface antigen with positive/negative anti-hepatitis B core (anti-HBc) antibody status in serum and this phenomenon is named occult hepatitis B infection (OBI). The presence of anti-HBc antibody in serum is an important key for OBI tracking, although about 20% of OBI cases are negative for anti-HBc antibody. The diagnosis of OBI is mainly based on polymerase chain reaction (PCR) and real-time PCR assays...
October 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27816852/prevention-of-inhibitor-development-in-hemophilia-a-in-2016-a-glimpse-into-the-future
#19
Massimo Franchini, Giuseppe Lippi
Thanks to considerable progresses made over the last 30years, hemophilia benefits from the most efficacious and safe treatment among the many monogenic inherited disorders. The most challenging complication of replacement therapy in hemophilia A is the occurrence of alloantibodies against infused factor VIII (FVIII), thus predisposing the patients to increased morbidity and disability. Extensive research in this field has definitively unraveled that development of inhibitors in hemophilia A is a complex and multifactorial process, in which inherited and environmental factors dynamically interact...
December 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27812261/clinical-characteristics-and-prognostic-factors-in-hemophiliacs-with-intracranial-hemorrhage-a-single-center-retrospective-experience
#20
Jung Yoon Cho, Won Seok Lee, Young Shil Park, Seung Hwan Lee, Jun Seok Koh
Intracranial hemorrhage (ICH) is the most serious bleeding event that occurs in patients with hemophilia; its estimated mortality rate is approximately 20 %, accounting for the largest number of deaths from bleeding. We conducted this single-center, retrospective study to examine the characteristics of and prognostic factors in patients with hemophilia. A comprehensive review of 12 cases of intracranial hemorrhage (ICH) among 10 patients. All 12 cases of ICH in the 10 patients were treated with clotting factor concentrates...
December 2016: Indian Journal of Hematology & Blood Transfusion
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