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hemophilia review

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https://www.readbyqxmd.com/read/28919830/identification-of-patients-with-congenital-hemophilia-in-a-large-electronic-health-record-database
#1
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/28895303/coronary-angiography-with-or-without-percutaneous-coronary-intervention-in-patients-with-hemophilia-systematic-review
#2
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
#3
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
#4
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/28874064/the-association-of-aging-with-von-willebrand-factor-levels-and-bleeding-risk-in-type-1-von-willebrand-disease
#5
Craig D Seaman, Margaret V Ragni
Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28838728/perioperative-use-of-coagulation-factor-concentrates-in-patients-undergoing-cardiac-surgery
#6
REVIEW
Mark M Smith, Elena Ashikhmina, Nathan J Brinkman, David W Barbara
Coagulopathy and bleeding are common in patients undergoing cardiac surgery, with a perioperative transfusion rate in excess of 50%. The mechanism of coagulopathy associated with cardiac surgery using cardiopulmonary bypass is multifactorial. Historically, coagulation factor-mediated bleeding in such instances has been treated with allogeneic plasma transfusion. Coagulation factor concentrate use for treatment of hemophilia, congenital factor deficiencies and, more recently, emergency warfarin reversal is common...
May 10, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28795008/sports-participation-recommendations-for-patients-with-bleeding-disorders
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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...
September 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
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