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haemophilia A review

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https://www.readbyqxmd.com/read/29741299/methodologies-for-data-collection-in-congenital-haemophilia-with-inhibitors-chwi-critical-assessment-of-the-literature-and-lessons-learned-from-recombinant-factor-viia
#1
REVIEW
C M Kessler, S Benchikh El Fegoun, A Worster
AIMS: To systematically review the effectiveness of on-demand treatment with recombinant coagulation factor VIIa (rFVIIa) in congenital haemophilia with inhibitors and, if feasible, perform a meta-analysis of the data. MATERIALS AND METHODS: Publications from Embase® , MEDLINE® , MEDLINE® In-Process and the Cochrane Central Register of Controlled Trials were searched. Selected publications were reviewed for inclusion by two independent expert reviewers. Discrepancies were reconciled by a third independent reviewer...
May 9, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29707874/total-elbow-arthroplasty-in-haemophilia
#2
REVIEW
T M Dale, J M Saucedo, E C Rodriguez-Merchan
INTRODUCTION: Advanced haemophilic arthropathy of the elbow can be a devastating manifestation of severe haemophilia with significant implications for activities of daily living. AIM: To summarize the currently available data on total elbow arthroplasty (TEA) for haemophilic arthropathy of the elbow, to draw comparisons to TEA for other indications, and to outline some considerations regarding TEA in this unique population. METHODS: A narrative review of the literature has been performed...
April 30, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29703220/european-principles-of-inhibitor-management-in-patients-with-haemophilia
#3
P L F Giangrande, C Hermans, B O'Mahony, P de Kleijn, M Bedford, A Batorova, J Blatný, K Jansone
BACKGROUND: In spite of recent major advances in the understanding and treatment of inhibitor development in patients with haemophilia, multidisciplinary management of many of these patients remains suboptimal and highly heterogenous across Europe. METHODS: Following a series of multidisciplinary meetings and a review of the literature, the European haemophilia community of health professionals and patients jointly defined practical optimum standards for ensuring and harmonizing treatment and care for patients with an inhibitor...
April 27, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29665204/factor-viii-products-and-inhibitor-development-in-previously-treated-patients-with-severe-or-moderately-severe-haemophilia-a-a-systematic-review
#4
Shermarke Hassan, Antonino Cannavò, Samantha Gouw, Frits R Rosendaal, Johanna van der Bom
BACKGROUND: Patients with severe haemophilia A who have been treated extensively with factor VIII (FVIII) products face a low but potentially serious risk of inhibitor development. It is unknown why these patients break immunological tolerance and data on product-related immunogenicity is scarce. AIMS: To summarize the currently available evidence on the relationship between inhibitor development and recombinant FVIII product type in previously treated patients with severe haemophilia A...
April 17, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29517971/non-factor-replacement-therapy-for-haemophilia-a-current-update
#5
REVIEW
Massimo Franchini, Pier Mannuccio Mannucci
One of the most challenging issues facing us in the treatment of haemophilia is the development of alloantibodies against infused factor VIII (FVIII) or factor IX (FIX). Inhibitors render factor replacement therapy ineffective, exposing patients to an unacceptably high risk of morbidity and mortality. Besides the well-known bypassing agents (i.e. activated prothrombin complex concentrate and recombinant activated factor VII) used to treat or prevent bleeding in haemophilia patients with inhibitors, there is growing interest in a new class of therapeutic agents which act by enhancing coagulation (i...
February 14, 2018: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29500556/correction-to-lonoctocog-alfa-a-review-in-haemophilia-a
#6
Zaina T Al-Salama, Lesley J Scott
The article Lonoctocog Alfa: A Review in Haemophilia A, written by Zaina T. Al-Salama and Lesley J. Scott, was originally published Online First without open access. After publication in volume 77, issue 15, pages 1677-1686 CSL Behring GmbH requested that the article be Open Choice to make the article an open access publication. Post-publication open access was funded by CSL Behring GmbH. Further details may be found at http://www.medengine.com/Redeem/CBD8F060224F2E65 . The article is forthwith distributed under the terms of the Creative Commons Attribution-NonCommercial 4...
March 2018: Drugs
https://www.readbyqxmd.com/read/29446525/laboratory-testing-for-factor-viii-and-ix-inhibitors-in-haemophilia-a-review
#7
REVIEW
C H Miller
Inhibitors are antibodies directed against haemophilia treatment products which interfere with their function. Factor VIII (FVIII) inhibitors in haemophilia A and factor IX (FIX) inhibitors in haemophilia B are significant clinically when they require a change in a patient's treatment regimen. Their persistence may increase morbidity and mortality. Multiple laboratory tests are now available for detecting and understanding inhibitors in haemophilia. Inhibitors are traditionally measured by their interference in clotting or chromogenic factor assays...
March 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436077/recombinant-factor-viii-fc-fusion-protein-for-immune-tolerance-induction-in-patients-with-severe-haemophilia-a-with-inhibitors-a-retrospective-analysis
#8
M Carcao, A Shapiro, J M Staber, N Hwang, C Druzgal, K Lieuw, M Belletrutti, C D Thornburg, S P Ahuja, J Morales-Arias, J Dumont, G Miyasato, E Tsao, N Jain, S W Pipe
INTRODUCTION: Immune tolerance induction (ITI) is the gold standard for eradication of factor VIII inhibitors in severe haemophilia A; however, it usually requires treatment for extended periods with associated high burden on patients and healthcare resources. AIM: Review outcomes of ITI with recombinant factor VIII Fc fusion protein (rFVIIIFc) in patients with severe haemophilia A and high-titre inhibitors. METHODS: Multicentre retrospective chart review of severe haemophilia A patients treated with rFVIIIFc for ITI...
March 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29405496/clinical-use-of-recombinant-factor-viii-fc-and-recombinant-factor-ix-fc-in-patients-with-haemophilia-a-and-b
#9
C Wang, G Young
INTRODUCTION: Although clinical trials have demonstrated extended half-life (EHL) VIII and IX fusion proteins to be safe and efficacious in patients with haemophilia A and B, studies on real-world clinical application have not been performed. AIM: To retrospectively examine the real-world experience of rFVIII Fc and rFIX Fc in patients. METHODS: A retrospective review of existing medical records of patients with haemophilia A or haemophilia B who had been prescribed rFVIII Fc or rFIX Fc was conducted from the Children's Hospital Los Angeles Haemostasis and Thrombosis Centre database...
February 5, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29363389/real-world-analysis-of-dispensed-ius-of-coagulation-factor-ix-and-resultant-expenditures-in-hemophilia-b-patients-receiving-standard-half-life-versus-extended-half-life-products-and-those-switching-from-standard-half-life-to-extended-half-life-products
#10
Bartholomew J Tortella, José Alvir, Margaret McDonald, Dean Spurden, Patrick F Fogarty, Amit Chhabra, Andreas M Pleil
BACKGROUND: Hemophilia B requires replacement therapy with factor IX (FIX) coagulation products to treat and prevent bleeding episodes. A recently introduced extended half-life (EHL) recombinant FIX replacement product provided the opportunity to compare the amount of dispensed factor and expenditures for EHL treatment compared with a standard half-life (SHL) product. OBJECTIVE: To determine factor international units (IUs) dispensed and expenditures associated with switching from nonacog alfa, the most commonly used SHL replacement product, to eftrenonacog alfa, an EHL FIX replacement product...
January 24, 2018: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/29359417/real-world-experience-with-use-of-antihemophilic-factor-recombinant-pegylated-for-prophylaxis-in-severe-haemophilia-a
#11
A L Dunn, S P Ahuja, E S Mullins
INTRODUCTION: Prophylaxis with extended half-life factor VIII (FVIII) is approved for haemophilia A, but data regarding routine clinical use are limited. AIM: To assess real-world experience of ADYNOVATE® (Antihemophilic Factor (Recombinant), PEGylated prophylaxis in children and adults with haemophilia A. METHODS: A retrospective chart review was conducted in three US haemophilia treatment centres. Records of all patients who began Adynovate prophylaxis in routine clinical practice were identified...
January 23, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29355793/position-of-the-french-working-group-on-perioperative-haemostasis-gihp-on-viscoelastic-tests-what-role-for-which-indication-in-bleeding-situations
#12
Stéphanie Roullet, Emmanuel de Maistre, Brigitte Ickx, Normand Blais, Sophie Susen, David Faraoni, Delphine Garrigue, Fanny Bonhomme, Anne Godier, Dominique Lasne
PURPOSE: Viscoelastic tests (VETs), thromboelastography (TEG® ) and thromboelastometry (ROTEM® ) are global tests of coagulation performed on whole blood. They evaluate the mechanical strength of a clot as it builds and develops after coagulation itself. The time required to obtain haemostasis results remains a major problem for clinicians dealing with bleeding, although some teams have developed a rapid laboratory response strategy. Indeed, the value of rapid point-of-care diagnostic devices such as VETs has increased over the years...
February 3, 2018: Anaesthesia, Critical Care & Pain Medicine
https://www.readbyqxmd.com/read/29328905/diagnosis-and-care-of-patients-with-mild-haemophilia-practical-recommendations-for-clinical-management
#13
REVIEW
Gary Benson, Günter Auerswald, Gerry Dolan, Anne Duffy, Cedric Hermans, Rolf Ljung, Massimo Morfini, Silva Zupančić Šalek
Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it may not present for many years after birth, and that awareness, even within families, may be low. Methodological problems exist in diagnosis, such as inconsistencies in results obtained from different assays used to measure factor levels in mild haemophilia. Advances in genetic testing provide insight into diagnosis as well as the likelihood of inhibitor development, which is not uncommon in patients with mild or moderate haemophilia and can increase morbidity...
November 14, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29282815/prevalence-of-and-risk-factors-for-cerebral-microbleeds-among-adult-patients-with-haemophilia-a-or-b
#14
H Husseinzadeh, T Chiasakul, P A Gimotty, B Pukenas, R Wolf, M Kelty, E Chiang, P F Fogarty, A Cuker
INTRODUCTION: Cerebral microbleeds (CMBs) represent clinically silent haemorrhagic events. Cerebral microbleeds (CMBs) portend negative neurovascular and cognitive outcomes in the general population and are associated with cognitive impairment in persons with haemophilia (PWH). Prevalence, patterns, and risk factors for CMBs in PWH have not been directly compared to persons without coagulopathy. AIM: To examine prevalence, patterns, and risk factors for CMBs in PWH vs normal controls...
March 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29212111/pathophysiological-mechanisms-of-endogenous-fviii-release-following-strenuous-exercise-in-non-severe-haemophilia-a-review
#15
C L Venema, R E G Schutgens, K Fischer
Introduction Non-severe haemophilia A is characterized by coagulation Factor VIII activity (FVIII:C) levels of 1 to 40 IU/dL. It has been reported that strenuous exercise increases the plasma FVIII:C in haemophilia A patients. This review highlights current knowledge about the pathophysiological mechanisms of endogenous FVIII release following strenuous exercise. Methods A literature search was performed to include relevant studies with data on pathophysiological mechanisms of FVIII release following strenuous exercise in haemophilia...
December 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29205699/use-of-telehealth-in-the-delivery-of-comprehensive-care-for-patients-with-haemophilia-and-other-inherited-bleeding-disorders
#16
REVIEW
R Kulkarni
Advances in technology such as telemedicine (TM) have made access to cost-effective, quality health care feasible for remote patients. TM is especially well suited for patients with chronic disorders such as haemophilia and related haemostatic disorders that benefit not only from more frequent interaction with care providers at a specialized haemophilia treatment center but also from consultations with other specialists. Telehealth refers to a broader application of TM and includes non-clinical services such as education, provider training, administrative meetings etc...
January 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29178149/measuring-activities-and-participation-in-persons-with-haemophilia-a-systematic-review-of-commonly-used-instruments
#17
REVIEW
M A Timmer, S C Gouw, B M Feldman, A Zwagemaker, P de Kleijn, M F Pisters, R E G Schutgens, V Blanchette, A Srivastava, J A David, K Fischer, J van der Net
INTRODUCTION: Monitoring clinical outcome in persons with haemophilia (PWH) is essential in order to provide optimal treatment for individual patients and compare effectiveness of treatment strategies. Experience with measurement of activities and participation in haemophilia is limited and consensus on preferred tools is lacking. AIM: The aim of this study was to give a comprehensive overview of the measurement properties of a selection of commonly used tools developed to assess activities and participation in PWH...
November 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29175823/major-bleeding-disorders-diagnosis-classification-management-and-recent-developments-in-haemophilia
#18
REVIEW
Neha Bhatnagar, Georgina W Hall
In this review, we outline the standard of care for children in the UK with the most common major bleeding disorder, haemophilia, and how exciting new developments in therapy have the potential for further improvements in quality of life and clinical outcome. The combination of comprehensive specialist medical care, safer factor concentrates, earlier introduction of prophylaxis and patient-specific education has allowed the current generation of patients with haemophilia to grow into adulthood with excellent joint function, pursuing full-time employment with a good quality of life...
November 25, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29172843/intra-articular-corticosteroid-injections-in-haemophilic-arthropathy-are-they-recommended
#19
REVIEW
E Carlos Rodriguez-Merchan
INTRODUCTION: Intra-articular (IA) corticosteroid (CS) injections are commonly used in the treatment of osteoarthritis. However, they are rarely utilized in haemophilic arthropathy. In fact, the efficacy of this method in haemophilic arthropathy is frequently discussed and debated in clinical practice. AIM: To investigate the effectiveness of IA CS injections in patients with painful haemophilic arthropathy. METHODS: A review of the literature on the topic was performed...
February 2018: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/29170896/modern-treatments-of-haemophilia-review-of-cost-effectiveness-analyses-and-future-directions
#20
REVIEW
Paolo A Cortesi, Lucia S D'Angiolella, Alessandra Lafranconi, Mariangela Micale, Giancarlo Cesana, Lorenzo G Mantovani
BACKGROUND: Cost is currently one of the most important aspects in haemophilia care. Factor concentrates absorb more than 90% of healthcare direct costs of haemophilia care, and the debate regarding the high cost of haemophilia treatments and their different use across different countries is increasing. OBJECTIVE: The objective of this study was to review cost-effectiveness analyses conducted on treatment options in haemophilia, focusing on their results and their strengths and limitations; to highlight the possible issues associated with economic evaluations of new treatment options...
March 2018: PharmacoEconomics
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