Choice of factor VIII/IX regimen in adolescents and young adults with severe or moderately severe haemophilia. A French national observational study (ORTHem 15-25).
Sign Up to like & getrecommendations! Published in 2017 at "Thrombosis research"
DOI: 10.1016/j.thromres.2016.12.023
Abstract: INTRODUCTION The value and challenges of long-term prophylaxis (LTP) in adolescents and young adults need further characterisation. AIM To determine the proportions of adolescents and young adults with severe or moderately severe haemophilia in France… read more here.
Keywords: adolescents young; treatment; young adults; factor viii ... See more keywords
How does a lifetime of painful experiences influence sensations and beliefs about pain in adults with severe haemophilia? A qualitative study.
Sign Up to like & getrecommendations! Published in 2021 at "Disability and rehabilitation"
DOI: 10.1080/09638288.2021.2018053
Abstract: PURPOSE To explore the life experiences of pain in people with severe haemophilia and understand how such experiences influence beliefs and sensation of pain in adulthood. METHODS A qualitative inquiry approach using focus groups and… read more here.
Keywords: severe haemophilia; experiences influence; pain; lifetime painful ... See more keywords
Treatment burden, haemostatic strategies and real world inhibitor screening practice in non‐severe haemophilia A
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DOI: 10.1111/bjh.14543
Abstract: Inhibitor formation in non‐severe haemophilia A is a life‐long risk and associated with morbidity and mortality. There is a paucity of data to understand real‐world inhibitor screening practice. We evaluated the treatment burden, haemostatic strategies,… read more here.
Keywords: risk; inhibitor; treatment; non severe ... See more keywords
Factor XIa‐triggered thrombin generation in severe haemophilia A
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DOI: 10.1111/bjh.15126
Abstract: Severe haemophilia A is associated with a severe bleeding phenotype (Richards et al, 2010). The factor VIII (FVIII) level is the mainstay of investigation and monitoring for these patients. However, there are often inconsistencies between… read more here.
Keywords: factor; generation; thrombin generation; triggered thrombin ... See more keywords
Advances in knowledge of inhibitor formation in severe haemophilia A
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DOI: 10.1111/bjh.16377
Abstract: Anti‐drug antibody formation following factor VIII (FVIII) replacement therapy is the most important treatment‐related complication in patients with severe haemophilia A. A significant number of these antibodies show neutralising activity against FVIII and are referred… read more here.
Keywords: knowledge inhibitor; formation severe; formation; advances knowledge ... See more keywords
rFVIII‐Fc in severe haemophilia A: The incentive switch in case of high risk of joint bleedings
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DOI: 10.1111/eci.13824
Abstract: Efmoroctocog alfa, the first recombinant factor VIII fusion protein with extended half‐life (rFVIII‐Fc), has been hypothesized to lower FVIII consumption in patients with severe Haemophilia A (pwSHA), without reducing clinical efficacy. What about real life? read more here.
Keywords: haemophilia incentive; incentive switch; switch case; severe haemophilia ... See more keywords
Pain and functional disability amongst adults with moderate and severe haemophilia from the Irish personalised approach to the treatment of haemophilia (iPATH) study
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DOI: 10.1111/ejh.13763
Abstract: To establish the prevalence of pain and functional disability in Irish adults with moderate and severe haemophilia, and to examine demographic and lifestyle influences. read more here.
Keywords: pain functional; functional disability; adults moderate; severe haemophilia ... See more keywords
Cost‐effectiveness analysis of late prophylaxis vs. on‐demand treatment for severe haemophilia A in Italy
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DOI: 10.1111/hae.13185
Abstract: Long‐term regular administrations of factor VIII (FVIII) concentrate (prophylaxis) initiated at an early age prevents bleeding in patients with severe haemophilia A (HA). The 5‐year prospective Italian POTTER study provided evidence of benefits in adolescents… read more here.
Keywords: late prophylaxis; effectiveness analysis; cost effectiveness; prophylaxis demand ... See more keywords
Modulation of the activated protein C pathway in severe haemophilia A patients: The effects of thrombomodulin and a factor V‐stabilizing fab
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DOI: 10.1111/hae.13300
Abstract: The thrombomodulin (TM)/activated protein C (APC) system is a key regulator of haemostasis, limiting amplification and propagation of the formed blood clot to the injury site. Dampening APC's inhibition of factor V (FV) and factor… read more here.
Keywords: protein pathway; modulation activated; activated protein; pathway severe ... See more keywords
Recombinant factor VIII Fc fusion protein for immune tolerance induction in patients with severe haemophilia A with inhibitors—A retrospective analysis
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DOI: 10.1111/hae.13413
Abstract: 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. read more here.
Keywords: tolerance induction; immune tolerance; factor viii; severe haemophilia ... See more keywords
Long‐term clinical and economic outcomes in previously untreated paediatric patients with severe haemophilia A: A nationwide real‐world study with 700 person‐years
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DOI: 10.1111/hae.13447
Abstract: For previously untreated patients (PUPs) with severe haemophilia A in Finland for the past 2 decades, the standard practice has been to start early primary prophylaxis. We evaluated the long‐term clinical outcomes and costs of… read more here.
Keywords: term clinical; long term; previously untreated; clinical economic ... See more keywords