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hemophilia review

Julie Ann Smith
Hemophilia will be encountered in the oral and maxillofacial surgeon's office. A thorough understanding of hemophilia is necessary to safely care for these patients. One must understand the severity of the patient's hemophilia as well as whether or not inhibitors are present. The patient's surgical management will be influenced by these two factors. In addition to the possible need to transfuse factors or desmopressin, special care must be taken perioperatively to avoid bleeding complications. This article reviews the overall management of hemophilia A and B as well as the specific perioperative management of these patients...
November 2016: Oral and Maxillofacial Surgery Clinics of North America
S M Johnson, G M Garnett, R K Woo
Maintenance of central venous access in patients with chronic medical conditions such as short bowel syndrome demands forethought and ingenuity. We describe an innovative technique for re-utilizing central venous access sites in patients who have chronic central venous access needs. Records of patients undergoing this technique were reviewed between August 2012 and December 2015. The technique involves "cutting-down" to the sterile fibrous tunnel that naturally forms around tunneled catheters. The fibrous sheath is then isolated and controlled much as would be done for a venous "cut-down...
October 14, 2016: Pediatric Surgery International
Peter S Fong, Devon M Flynn, Christopher D Evans, P Todd Korthuis
Integrase strand transfer inhibitors (INSTIs) have become integral antiretroviral therapy (ART) agents for treating HIV infection. We report the case of a 44-year-old male with a history of hemophilia A who developed diabetes mellitus four months after switching from abacavir, lamivudine, and efavirenz to abacavir, lamivudine, and raltegravir. Hemoglobin A1C normalized without further need for exogenous insulin after raltegravir was switched back to efavirenz. In this case report, we will review a possible mechanism for INSTI-induced hyperglycemia and/or diabetes mellitus...
October 12, 2016: International Journal of STD & AIDS
E Carlos Rodriguez-Merchan
INTRODUCTION: It has been reported an increase of angiogenesis and vascular remodeling in hemophilic arthropathy. Therefore, antiangiogenic drugs could be useful in the management of hemophilic arthropathy. The aim of this review paper is to revise the role of angiogenesis in hemophilic arthropathy. AREAS COVERED: This article is a narrative review of the pathophysiology of the disturbed angiogenesis in hemophilia and the potential role of antiangiogenic drugs in nonhemophilia patients Expert commentary: Antiangiogenic drugs could be helpful in the future for the treatment of hemarthroses in hemophilic arthropathy...
October 2016: Expert Review of Hematology
Asim Azhar, Ejaj Ahmad, Qamar Zia, Mohammad Owais, Ghulam Md Ashraf
Therapeutic proteins are engineered proteins produced in the laboratory for pharmaceutical use. With the advent of recombinant DNA technology, the proteins can be generated in specific host cells under defined conditions. In the process of production of genetically engineered animals, the gene of interest can be added at a single cell stage to produce a cloned animal from genetically engineered cells. Several recombinant cytokines, clotting factors etc have been licensed and are currently being utilized for the treatment of cancer, infectious diseases, hemophilia, anemia, multiple sclerosis, and hepatitis B/C...
September 1, 2016: Current Protein & Peptide Science
Rowena C Punzalan, Jerome L Gottschall
Although congenital bleeding disorders can manifest in the newborn period, the most common causes of bleeding and thrombosis in neonates are acquired conditions. Factor concentrates are used for specific diagnoses (hemophilia with inhibitors, specific factor deficiency, von Willebrand disease) and approved indications, and increasingly for off-label indications (bleeding in surgery cardiopulmonary bypass, extracorporeal membrane oxygenation). We will review the approved indications for factor products in the neonate and discuss the evidence and rationale for off-label use of factor products in management of bleeding and thrombosis in the neonate...
October 2016: Transfusion Medicine Reviews
Jan Hartmann, Stacy E Croteau
A surge in therapeutic clinical trials over recent years is paving the way for transformative treatment options for patients with hemophilia. The introduction of recombinant factor concentrates in the early 1990s facilitated the use of prophylactic replacement as standard care for hemophilia rather than on-demand treatment. This has revolutionized health outcomes for hemophilia patients, enabling participation in physical activities and reducing debilitating, chronic joint damage. Challenges of prophylactic factor infusion include the frequency of infusions needed to maintain factor levels greater than 1%, patient adherence, reliable intravenous access, and development of neutralizing alloantibodies ("inhibitors")...
August 26, 2016: American Journal of Hematology
Alanna McEneny-King, Alfonso Iorio, Gary Foster, Andrea N Edginton
INTRODUCTION: Hemophilia A is a bleeding disorder resulting from a lack of clotting factor VIII (FVIII), and treatment typically consists of prophylactic replacement of the deficient factor. However, high between subject variability precludes the development of a 'one size fits all' dosing strategy and necessitates an individualized approach. We sought to summarize the data on the pharmacokinetics of FVIII available as a basis for the development of population pharmacokinetic models to be used in dose tailoring...
August 19, 2016: Expert Opinion on Drug Metabolism & Toxicology
Tian-Meng Yan, Chun-Xia He, Bao-Lai Hua, Li Li, Hong-Zhong Jin, Yue-Hua Liu, Ya-Gang Zuo
Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients...
August 11, 2016: Journal of Dermatology
A Iorio, A M Barbara, F Bernardi, D Lillicrap, M Makris, F Peyvandi, F Rosendaal
Aim The scope of this recommendation is to provide guidance for reporting of inhibitor cases in previously treated patients (PTPs) with hemophilia A. This guidance is intended to improve transparency and completeness of reporting of observed events; it does not cover planning, executing or analyzing original studies aimed at the assessment of inhibitor rates. Recommendation We recommend that for each case of inhibitor development reported in a published paper, a paragraph or a table is included in the main publication reporting as a minimum the underlined data fields in Table ...
August 2016: Journal of Thrombosis and Haemostasis: JTH
Jennifer Pocoski, Nanxin Li, Rajeev Ayyagari, Nikki Church, Monika Maas Enriquez, Quer Xiang, Sneha Kelkar, Ella X Du, Eric Q Wu, Jipan Xie
BACKGROUND: No head-to-head trials comparing recombinant factor VIII (rFVIII) products currently exist. This was a matching-adjusted indirect comparison (MAIC) study of efficacy of BAY 81-8973 with antihemophilic factor (recombinant) plasma/albumin-free method (rAHF-PFM) and turoctocog alfa for the prophylaxis of severe hemophilia A. METHODS: A systematic literature review was conducted to identify trials of rAHF-PFM and turoctocog alfa. Comparisons were conducted using BAY 81-8973 individual patient data (IPD) from LEOPOLD trials and published data from rAHF-PFM and turoctocog alfa trials...
2016: Journal of Blood Medicine
Ramon Mohanlal, Yuhong Qiu, Ming Zheng, Asmae Mirkou, Kanaka Sridharan, Christopher Keir
OBJECTIVE: Our objective was to evaluate the life-long safety profile of gene therapy using retroviral (non-replicating) vectors (nRCR), or cell products in 127 subjects with hemophilia, human immunodeficiency virus (HIV), or cancer, previously treated with such gene therapy. METHODS: We assessed the occurrence of serious adverse events (SAEs), deaths and presence of replication competent retrovirus (RCR). RESULTS: A total of 23 subjects remained until the data cut-off date of 31 July 2013 and provided safety information of up to 18 years...
July 19, 2016: Molecular Diagnosis & Therapy
John Chapin, Jaqueline Bamme, Fraustina Hsu, Paul Christos, Maria DeSancho
Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed...
July 14, 2016: Clinical and Applied Thrombosis/hemostasis
Yan Zeng, Ruiqing Zhou, Xin Duan, Dan Long
BACKGROUND: Acquired haemophilia A is a rare bleeding disorder caused by the development of specific autoantibodies against coagulation factor VIII. Rituximab may be an alternative approach to the treatment of acquired haemophilia by eradicating FVIII autoantibodies. OBJECTIVES: To assess and summarise the efficacy and adverse effects of rituximab for treating people with acquired haemophilia A. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's trials registers, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings...
2016: Cochrane Database of Systematic Reviews
Hong Yan, Fanyi Zeng
Hemophilia B is an X chromosome linked hereditary hemorrhagic disease, which is caused by the lose function mutation of factor IX (FIX), and significantly affects the patients' lifespan and life quality. The severity of hemophilia B depends on the FIX level in the plasma. By referring to the relevant literatures, we reviewed and summarized hemophilia B replacement therapies. Specifically, we focus on recombinant factor IX products on the market and those in the pipeline, especially on the long-acting factor IX drugs, to provide the basis for researches of new hemophilia B drugs...
February 2016: Sheng Wu Gong Cheng Xue Bao, Chinese Journal of Biotechnology
Amana Farrkh, Emilia Garrison, James J Closmann
The dental surgical management of a patient with a bleeding disorder can present a challenge to the dental provider. This article reviews the clinical presentation of hemophilia and describes the management of 2 patients with hemophilia who presented for dental extractions. Each patient's hematologist was consulted to determine clotting factor replacement therapy, antifibrinolytic therapy, and local measures to obtain hemostasis perioperatively. Careful coordination with the patient's hematologist in such cases is essential to ensure safe treatment...
July 2016: General Dentistry
Shamsoreza Tabriznia-Tabrizi, Marzie Gholampour, Hassan Mansouritorghabeh
INTRODUCTION: Hemophilia A (HA) has an X-linked pattern of inheritance and is the most common of the hemorrhagic disorders. HA is caused by a decreased or deficiency of the functional clotting factor VIII (FVIII) and effects 1 in 5000-10,000 male births. The common treatment for hemophilia is replacement therapy by plasma-derived or recombinant FVIII. Approximately 20-30% of people with a severe type of HA develop an inhibitor and this phenomenon is the main challenge in the management of these patients...
September 2016: Expert Review of Hematology
P Maxwell Courtney, Blair S Ashley, Eric L Hume, Atul F Kamath
BACKGROUND: Alternative payment models, such as the Centers for Medicare & Medicaid Services (CMS) Bundled Payment for Care Improvement (BPCI) initiative, aim to decrease overall costs for hip and knee arthroplasties. QUESTIONS/PURPOSES: We asked: (1) Is there any difference in the CMS episode-of-care costs, hospital length of stay, and readmission rate from before and after implementation of our bundled-payment program? (2) Is there any difference in reimbursements and resource utilization between revision THA and TKA at our institution? (3) Are there any independent risk factors for patients with high costs who may not be appropriate for a bundled-payment system for revision total joint arthroplasty (TJA)? METHODS: Between October 2013 and March 2015, 218 patients underwent revision TKA or THA in one health system...
June 29, 2016: Clinical Orthopaedics and related Research
M Pai, N Santesso, C H T Yeung, S J Lane, H J Schünemann, A Iorio
BACKGROUND: Rigorous and transparent methods are necessary to develop clinically relevant and evidence-based practice guidelines. We describe the development of the National Hemophilia Foundation-McMaster Guideline on Care Models for Haemophilia Management, which addresses best practices in haemophilia care delivery. METHODS: We assembled a Panel of persons with haemophilia (PWH), parents of PWH, clinical experts and guideline methodologists. Conflicts of interest were disclosed and managed throughout...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Victoria H Wilkinson, Emma L Rowbotham, Andrew J Grainger
The foot and ankle are commonly involved in a range of arthritides that affect the joints, bones, and soft tissues. Accurate plain film interpretation can often aid the diagnosis and monitor disease progression and treatment response. Ultrasound and MRI afford superior depiction of the soft tissues, and advances over recent years have centered on early detection of synovitis, enabling earlier diagnosis and treatment. Advantages and disadvantages of the imaging techniques of radiography, multidetector computed tomography, ultrasound, and MRI are discussed, as is optimization of these modalities for the assessment of the anatomically complex joints of the foot and ankle...
April 2016: Seminars in Musculoskeletal Radiology
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