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https://www.readbyqxmd.com/read/28795008/sports-participation-recommendations-for-patients-with-bleeding-disorders
#1
REVIEW
Cailly Howell, Katherine Scott, Dilip R Patel
There are several sets of guidelines recommending sports participation for patients with bleeding disorders. Though there is a perceived risk of injury and the associated bleeding complications, several studies have been done in this patient population and have found no increased risk. In addition, there are many other benefits to participation including improvement in physical fitness status, better health outcomes, improvement in quality of life, and the development of increased flexibility, gait coordination, and muscular strength, which may actually reduce the risk of subsequent injury...
July 2017: Translational pediatrics
https://www.readbyqxmd.com/read/28769599/identification-of-people-with-acquired-hemophilia-in-a-large-electronic-health-record-database
#2
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) can provide insights into diagnoses, treatment patterns, and clinical outcomes. Acquired hemophilia (AH) is an ultrarare bleeding disorder characterized by factor VIII inhibiting autoantibodies. AIM: To identify patients with AH using an EHR database. METHODS: Records were accessed from a large EHR database (Humedica) between January 1, 2007 and July 31, 2013. Broad selection criteria were applied using the International Classification of Diseases, Ninth Revision, clinical modification (ICD-9-CM) code for intrinsic circulating anticoagulants (286...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28758960/ultrasound-for-early-detection-of-joint-disease-in-patients-with-hemophilic-arthropathy
#3
REVIEW
Matteo Nicola Dario Di Minno, Gianluigi Pasta, Sonia Airaldi, Federico Zaottini, Antonio Storino, Ernesto Cimino, Carlo Martinoli
Joint bleeding represents the most commonly reported type of hemorrhage in patients affected by hemophilia. Although the widespread use of prophylaxis has been able to significantly reduce the onset of arthropathy, it has been shown that a non-negligible percentage of patients develop degenerative changes in their joints despite this type of treatment. Thus, periodic monitoring of the joint status in hemophilia patients has been recommended to identify early arthropathic changes and prevent the development or progression of hemophilic arthropathy...
July 31, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28725275/ash-meeting-2016-developments-in-hemostaseology
#4
REVIEW
Clemens Feistritzer, Birgit Mosheimer
During the annual meeting of the American Society of Hematology (ASH) in San Diego/California, novel developments in the field of hemostaseology were presented. Alternative treatment strategies besides factor replacement were discussed for patients with hemophilia. One of the highlights of the meeting in this year's plenary session was the presentation of successful adeno-associated virus mediated gene transfer in patients with hemophilia B leading to sustained elevation of factor IX:C (FIX:c). Other alternative treatment approaches in patients with hemophilia A may include bispecific antibodies mimicking factor VIIIa (FVIIIa) activity or disrupting anticoagulant proteins...
2017: Memo
https://www.readbyqxmd.com/read/28724345/simultaneous-bilateral-total-knee-arthroplasty-in-hemophilia-is-it-recommended
#5
E Carlos Rodriguez-Merchan
Simultaneous bilateral total knee arthroplasty (TKA) is a potentially cost-saving manner of caring for patients with hemophilia who have bilateral symptomatic hemophilic knee arthropathy. Because factor VIII is used only once in bilateral TKA, it is performed in hemophilia to reduce costs. Areas covered: This article reviews the potential role of simultaneous bilateral TKA in patients with hemophilia. Expert commentary: Although two recent reports have mentioned the benefits of simultaneous bilateral TKA in patients with hemophilia, the data are still scant and preliminary...
July 20, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28716211/to-serve-and-protect-the-modulatory-role-of-von-willebrand-factor-on-factor-viii-immunogenicity
#6
REVIEW
Robin B Hartholt, Alice S van Velzen, Ivan Peyron, Anja Ten Brinke, Karin Fijnvandraat, Jan Voorberg
Hemophilia A is a bleeding disorder characterized by the absence or dysfunction of blood coagulation factor VIII (FVIII). Patients are treated with regular infusions of FVIII concentrate. In response to treatment, approximately 30% of patients with severe hemophilia A develop inhibitory antibodies targeting FVIII. Both patient and treatment related risk factors for inhibitor development have been described. Multiple studies comparing the immunogenicity of recombinant and plasma-derived FVIII have yielded conflicting results...
July 4, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28692575/rendering-factor-xa-zymogen-like-as-a-therapeutic-strategy-to-treat-bleeding
#7
Nabil K Thalji, Rodney M Camire
PURPOSE OF REVIEW: New therapies are needed to control bleeding in a range of clinical conditions. This review will discuss the biochemical properties of zymogen-like factor Xa, its preclinical assessment in different model systems, and future development prospects. RECENT FINDINGS: Underlying many procoagulant therapeutic approaches is the rapid generation of thrombin to promote robust clot formation. Clinically tested prohemostatic agents (e.g., factor VIIa) can provide effective hemostasis to mitigate bleeding in hemophilia and other clinical situations...
September 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28691218/safety-of-a-pasteurized-plasma-derived-factor-viii-and-von-willebrand-factor-concentrate-analysis-of-33-years-of-pharmacovigilance-data
#8
Peter Kouides, Kathrin Wawra-Hehenberger, Anna Sajan, Henry Mead, Toby Simon
BACKGROUND: Haemate-P/Humate-P (Humate-P) is a pasteurized human plasma-derived concentrate containing both Factor VIII and von Willebrand factor for treatment of hemophilia A and von Willebrand disease (VWD). STUDY DESIGN AND METHODS: We analyzed the safety of Humate-P based on more than 33 years of postmarketing pharmacovigilance data, representing an estimated exposure of approximately 25,000 patient-years. The analysis comprises reports of potential adverse drug reactions (ADRs) from all sources, reported as part of routine pharmacovigilance at CSL Behring...
July 10, 2017: Transfusion
https://www.readbyqxmd.com/read/28685500/rituximab-for-treating-inhibitors-in-people-with-inherited-severe-hemophilia
#9
REVIEW
Lucan Jiang, Yi Liu, Lingli Zhang, Cristina Santoro, Armando Rodriguez
BACKGROUND: Hemophilia A and B are inherited coagulation disorders characterized by a reduced or absent level of factor VIII or factor IX respectively. The severe form is characterized by a factor level less than 0.01 international units (IU) per milliliter. The development of inhibitors in hemophilia is the main complication of treatment, because the presence of these antibodies, reduces or even nullifies the efficacy of replacement therapy, making it very difficult to control the bleeding...
July 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28681633/major-and-minor-classifications-for-surgery-in-people-with-hemophilia-a-literature-review
#10
Luigi Piero Solimeno, Miguel A Escobar, Snejana Krassova, Stephanie Seremetis
Agents that control bleeding and the usage of bypassing agents have made surgery an option to consider in people with hemophilia. However, the lack of consistent definitions for major or minor surgery may lead to inconsistencies in patient management. This literature review has evaluated how surgical procedures in people with hemophilia were categorized as major or minor surgery and assessed the consistency across publications. After screening 926 potentially relevant articles, 547 were excluded and 379 full-text articles were reviewed...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28670147/many-factor-viii-products-available-in-the-treatment-of-hemophilia-a-an-embarrassment-of-riches
#11
REVIEW
Kenneth Lieuw
Hemophilia A (HA) is a common bleeding disorder caused by the deficiency of factor VIII (FVIII) with an incidence of ~1 in 5000 male births. Replacement of FVIII is necessary to prevent and treat bleeding episodes. However, with multiple new drugs in addition to old standards, choosing among the different FVIII treatment options is harder than ever. There are FVIII products that are plasma derived or recombinant, FVIII products designed to extend the half-life of FVIII, and the first single-chain FVIII product, recombinant factor VIII single chain (rFVIII-SC)...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28668202/home-infusion-safe-clinically-effective-patient-preferred-and-cost-saving
#12
REVIEW
Jennifer M Polinski, Mary K Kowal, Michael Gagnon, Troyen A Brennan, William H Shrank
BACKGROUND: As the U.S. healthcare payment system shifts from volume to value, identifying care approaches that improve outcomes while lowering costs are essential. We sought to understand the utility of home infusion versus medical-setting infusion as a mechanism to affect the three-part aim: better care, better health outcomes, and lower costs. STUDY DESIGN: Systematic review. METHODS: We searched MEDLINE, EMBASE, and Science Citation Index for articles related to the safety, clinical effectiveness, quality of life and satisfaction, and/or costs of home infusion as compared with infusion in an outpatient medical facility or hospital...
March 2017: Healthcare
https://www.readbyqxmd.com/read/28662249/hemophilic-arthropathy
#13
Christopher R Nacca, Andrew P Harris, John R Tuttle
The musculoskeletal manifestations of hemophilia A and B are some of the most common presenting symptoms and continue to be challenging to practitioners. Hemophilic arthropathy, if not initially adequately treated and managed, may lead to debilitating disease and eventually require the consideration of major surgery, including total joint arthroplasty. Thorough comprehension of the pathophysiology, diagnosis, and both medical and surgical interventions is critical in establishing an appropriate treatment regimen for these patients...
June 29, 2017: Orthopedics
https://www.readbyqxmd.com/read/28651073/hemophilia-a-inhibitor-treatment-the-promise-of-engineered-t-cell-therapy
#14
REVIEW
Kalpana Parvathaneni, Maha Abdeladhim, Kathleen P Pratt, David W Scott
Hemophilia A is a bleeding disorder caused by mutations in the gene encoding factor VIII (FVIII), a cofactor protein that is essential for normal blood clotting. Approximately, 1 in 3 patients with severe hemophilia A produce neutralizing antibodies (inhibitors) that block its biologic function in the clotting cascade. Current efforts to eliminate inhibitors consist of repeated FVIII injections under what is termed an "ITI" protocol (Immune Tolerance Induction). However, this method is extremely costly and approximately 30% of patients undergoing ITI do not achieve peripheral tolerance...
June 9, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/28648539/prothrombin-complex-concentrates-in-pediatric-cardiac-surgery-the-current-state-and-the-future
#15
REVIEW
Elena Ashikhmina, Sameh Said, Mark M Smith, Vilmarie Rodriguez, William C Oliver, Gregory A Nuttall, Joseph A Dearani, Hartzell V Schaff
BACKGROUND: After decades of practice of pediatric cardiac surgery, postoperative bleeding due to the immaturity of hemostasis, hemodilution, and hypothermia remains a concern. Recently, a new approach for adult coagulopathy after bypass has emerged. Prothrombin complex concentrates (PCCs), designed to treat bleeding in hemophilia patients, are safely and efficiently used off label for hemorrhage after bypass. However, optimal dosing, indications and contraindications, and laboratory tests to assess the efficacy of PCC use in children have not yet been established...
June 22, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28645365/complexity-of-immune-responses-to-aav-transgene-products-example-of-factor-ix
#16
REVIEW
Roland W Herzog
After two decades of research, in vivo gene transfer with adeno-associated viral (AAV) vectors has now resulted in successful treatments and even cures for several human diseases. However, the potential for immune responses against the therapeutic gene products remains one of the concerns as this approach is broadened to more patients, diverse diseases, and target organs. Immune responses following gene transfer of coagulation factor IX (FIX) for the treatment of the bleeding disorder hemophilia B has been extensively investigated in multiple animal models...
May 29, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28641667/-research-progress-on-genetic-factors-and-hemophilia-a-clotting-factor-inhibitor-review
#17
Zheng-Bin Hu, Xin Sun, Li-Ya He
Hemophilia A (Hemophilia A, HA) is an X-linked recessive hereditary coagulation function disorder, the deficiency and dysfunction of blood coagulation were caused by the mutations of gene encoding clotting factor VIII. The treatment of hemophilia A still depends on the replacement therapy with blood coagulation factor. However, the repeated infusion of clotting factor will produce the neutralizing antibody against FVIII, then resulting in one of the serious complications. The reports on the incidence of inhibitor are different at home and abroad...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28599169/modifiers-of-clinical-phenotype-in-severe-congenital-hemophilia
#18
Massimo Franchini, Pier Mannuccio Mannucci
Patients with inherited hemophilia A and B usually exhibit a bleeding tendency of a severity proportional to the degree of plasmatic deficiency of the coagulant activity of factor VIII (FVIII:C) and factor IX (FIX:C). Although patients with severe hemophilia (i.e., with FVIII:C and FIX:C levels <1IU/dL) are generally those with the most severe bleeding phenotype, it is common experience that a variable proportion of them experiences a milder bleeding tendency. In this review, we summarize the current knowledge on the possible mechanisms at the basis of the phenotypic heterogeneity of severe hemophilia, focusing in particular on the role of FVIII/FIX gene mutations and thrombophilic polymorphisms...
June 1, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28589615/safety-and-efficacy-of-recombinant-activated-coagulation-factor-vii-in-congenital-hemophilia-with-inhibitors-in-the-home-treatment-setting-a-review-of-clinical-studies-and-registries
#19
REVIEW
Guy Young, Miguel A Escobar, Steven W Pipe, David L Cooper
Self-administration of factor and bypassing agents by persons with hemophilia in the home setting is recommended to facilitate earlier intervention after bleeding episodes. The objective of this review was to summarize recombinant activated coagulation factor VII (rFVIIa) safety and efficacy data from clinical trials and patient registries documenting use in the home treatment setting in people with congenital hemophilia with inhibitors (CHwI). A total of 16 studies and registries were identified for inclusion; 14 evaluated on-demand treatment of acute bleeding episodes (865 patients, 9024 bleeding episodes) and 2 evaluated use for secondary prophylaxis (108 patients, 42,861 prophylaxis days)...
September 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28586248/side-effects-and-potential-risk-factors-of-botulinum-toxin-type-a-intramuscular-injections-in-knee-flexion-contractures-of-hemophiliacs
#20
E Carlos Rodriguez-Merchan, Hortensia De la Corte-Rodriguez
Knee flexion contracture (KFC) is a common complication of recurrent hemarthrosis in children and young adults with hemophilia. If the KFC is not prevented (by means of primary prophylaxis) and treated properly and early (be means of physical medicine and rehabilitation), it will become fixed. Areas covered: The aim of this article is to review the potential role of botulinum toxin type A (BTX-A) intramuscular injections for the treatment of KFC in people with hemophilia (PWH). Expert commentary: Although two recent reports have mentioned the benefits of intramuscular injections of BTX-A in PWH with KFC, the data are still scant and too preliminary...
June 19, 2017: Expert Review of Hematology
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