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haemophilia A review

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https://www.readbyqxmd.com/read/29328905/diagnosis-and-care-of-patients-with-mild-haemophilia-practical-recommendations-for-clinical-management
#1
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
#2
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...
December 28, 2017: 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
#3
C L Venema, R E G Schutgens, K Fischer
No abstract text is available yet for this article.
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
#4
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...
December 5, 2017: 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
#5
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
#6
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
#7
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...
November 30, 2017: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/29170896/modern-treatments-of-haemophilia-review-of-cost-effectiveness-analyses-and-future-directions
#8
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...
November 23, 2017: PharmacoEconomics
https://www.readbyqxmd.com/read/29124682/nonacog-beta-pegol-a-review-in-haemophilia-b
#9
Yahiya Y Syed
Nonacog beta pegol [Refixia(®) (EU)] is an intravenously-administered, glycoPEGylated recombinant factor IX (FIX), with an extended terminal half-life. It is approved in the EU for the treatment and prophylaxis of bleeding in patients aged ≥ 12 years with haemophilia B. The therapeutic efficacy and safety of nonacog beta pegol was demonstrated in the phase 3 Paradigm trials in previously treated adolescents and adults with haemophilia B. In Paradigm 2, nonacog beta pegol showed good haemostatic effects when treating bleeds on-demand, and reduced annualized bleeding rates when used as a once-weekly prophylaxis...
November 9, 2017: Drugs
https://www.readbyqxmd.com/read/29051804/review-of-recombinant-anti-haemophilic-porcine-sequence-factor-viii-in-adults-with-acquired-haemophilia-a
#10
REVIEW
Emma Fosbury, Anja Drebes, Anne Riddell, Pratima Chowdary
Acquired haemophilia A (AHA) is a rare, serious bleeding disorder most often encountered in elderly patients. The mainstay of haemostatic management is with bypassing agents (BPAs) including recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCCs). Their major limitation is incomplete efficacy, potential risk for thrombosis and the lack of routine laboratory assays for monitoring treatment response. Plasma-derived porcine FVIII (pd-pFVIII, Hyate C(®)), first used in the 1950s for the management of congenital haemophilia, has sufficient sequence homology to be haemostatic in humans, but the lack of complete homology facilitates efficacy even in the presence of human allo- and autoantibodies against human FVIII (hFVIII)...
September 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28960809/acquired-haemophilia-in-cancer-a-systematic-and-critical-literature-review
#11
REVIEW
M Napolitano, S Siragusa, S Mancuso, C M Kessler
AIM: There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer. METHODS: Treatment outcomes of AH were defined as complete response (CR), partial response (PR) or no response (NR), based on inhibitor eradication, coagulation factor VIII levels and bleeding control. Reported deaths were either related to cancer or bleeding...
September 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28944952/bypassing-agent-prophylaxis-in-people-with-hemophilia-a-or-b-with-inhibitors
#12
REVIEW
Chatree Chai-Adisaksopha, Sarah J Nevitt, Mindy L Simpson, Maissaa Janbain, Barbara A Konkle
BACKGROUND: People with hemophilia A or B with inhibitors are at high risk of bleeding complications. Infusion of bypassing agents, such as recombinant activated FVII (rFVIIa) and plasma-derived activated prothrombin complex concentrate, are suggested as alternative therapies to factor VIII (haemophilia A) or IX (haemophilia B) for individuals who no longer respond to these treatments because they develop inhibitory antibodies. The ultimate goal of treatment is to preserve the individual's joints, otherwise destroyed by recurrent bleeds...
September 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28942686/fibrin-glue-for-local-haemostasis-in-haemophilia-surgery
#13
E Carlos Rodriguez-Merchan
INTRODUCTION: Local fibrin glue (FG) appears to be a useful local haemostatic agent for severe haemorrhage in people with haemophilia (PWH) undergoing surgical procedures. AIM: To evaluate the role of local FG in PWH. METHODS: A review of the literature on the topic has been performed. RESULTS: Local FG is not always necessary to achieve haemostasis in all surgical procedures performed in PWH. However, it could be a good adjunct therapy, primarily when a surgical field will bleed more than expected (e...
October 3, 2017: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/28900904/lonoctocog-alfa-a-review-in-haemophilia-a
#14
Zaina T Al-Salama, Lesley J Scott
Lonoctocog alfa (rVIII-SingleChain; Afstyla(®)) is a novel single-chain recombinant factor VIII (FVIII) molecule, with a truncated B-domain and the heavy and light chains covalently linked to form a stable and homogenous drug that binds with high affinity to von Willebrand factor (VWF). Intravenous lonoctocog alfa is approved for the prophylaxis and treatment of bleeding in patients with haemophilia A in several countries worldwide. In two pivotal, multicentre trials, lonoctocog alfa was effective in the treatment of bleeding episodes and as prophylaxis, including for perioperative management in adults, adolescents and children...
October 2017: Drugs
https://www.readbyqxmd.com/read/28876509/clot-waveform-analysis-where-do-we-stand-in-2017
#15
REVIEW
P O Sevenet, F Depasse
Analysis of the optical waveform generated during global coagulation assays, such as activated partial thromboplastin time and prothrombin time, can provide much precious information on the global coagulation state of the plasma sample tested, in addition to a single clotting time. Many studies have been published concerning patient diagnosis and management in haemophilia A, and in the early diagnosis and prognosis of disseminated intravascular coagulation and sepsis. However, many other works have also been published on further potential clinical applications such as lupus anticoagulant diagnosis and anticoagulant monitoring...
December 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28871021/effectiveness-of-two-psychological-interventions-for-pain-management-emotional-regulation-and-promotion-of-quality-of-life-among-adult-portuguese-men-with-haemophilia-psy-haemopeq-study-protocol-for-a-single-centre-prospective-randomised-controlled-trial
#16
Patrícia Ribeiro Pinto, Ana Cristina Paredes, Patrício Costa, Manuela Carvalho, Manuela Lopes, Susana Fernandes, Susana Pedras, Armando Almeida
INTRODUCTION: Haemophilia is a bleeding disorder associated with significant pain, emotional distress, quality of life (QoL) impairment and considerable healthcare costs. Psychosocial health and effective pain management are considered essential end points for optimal haemophilia care, but there is a significant gap in evidence-based treatments targeting these outcomes in people with haemophilia (PWH). Psychological interventions are cost-effective in promoting emotional well-being, QoL and pain control, although these have been scarcely used in haemophilia field...
September 3, 2017: BMJ Open
https://www.readbyqxmd.com/read/28838029/radionuclide-synovectomy-synoviorthesis-rs-in-patients-with-bleeding-disorders-a-review-of-patient-and-procedure-demographics-and-functional-outcomes-in-the-athndataset
#17
C McGuinn, D Cheng, D Aschman, S L Carpenter, R Sidonio, A Soni, M D Tarantino, A P Wheeler, A L Dunn
INTRODUCTION: Radionuclide synovectomy/synoviorthesis (RS) to manage proliferative synovitis in persons with bleeding disorders has been utilized for decades; however, aggregate US results are limited. AIM: To determine the prevalence of RS utilization, patient and procedure related demographics and functional outcomes in United States haemophilia treatment centres (HTCs). The ATHNdataset includes US patients with bleeding disorders who have authorized the sharing of their demographic and clinical information for research...
November 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28835123/advances-in-gene-therapy-for-hemophilia
#18
Amit C Nathwani, Andrew M Davidoff, Edward G D Tuddenham
Gene therapy provides hope for a cure for patients with hemophilia by establishing continuous endogenous expression of factor VIII or factor IX following transfer of a functional gene copy to replace the hemophilic patient's own defective gene. Hemophilia may be considered a "low-hanging fruit" for gene therapy because a small increment in blood factor levels (≥2% of normal) significantly improves the bleeding tendency from severe to moderate, eliminating most spontaneous bleeds. After decades of research, the first trial to provide clear evidence of efficiency after gene transfer in patients with hemophilia B using adeno-associated virus vectors was reported by the authors' group in 2011...
November 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28826659/haemophilia-b-where-are-we-now-and-what-does-the-future-hold
#19
REVIEW
Gerry Dolan, Gary Benson, Anne Duffy, Cedric Hermans, Victor Jiménez-Yuste, Thierry Lambert, Rolf Ljung, Massimo Morfini, Silva Zupančić Šalek
Research has been lacking on the natural history, complications, and treatment of haemophilia B, which is less common than haemophilia A and was recognized as a distinct clinical entity in 1947. Although the two diseases share the same clinical manifestations, they differ in causative mutation, risk of inhibitor development, and patient quality of life. Frequently debated is whether haemophilia B is as clinically severe as haemophilia A, with much of the published data on overall and haemophilia-specific health outcomes suggesting that haemophilia B may have a less severe clinical phenotype...
August 16, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28780770/subcutaneous-diphtheria-and-tetanus-vaccines-in-children-with-haemophilia-a-pilot-study-and-review-of-the-literature
#20
B A Schaefer, R A Gruppo, E S Mullins, C Tarango
INTRODUCTION: Subcutaneous (SQ) vaccination has emerged as standard of care in children with severe bleeding disorders to reduce unnecessary factor exposure and avoid provoking an intramuscular bleed, but little is known about comparative immunogenicity to intramuscular (IM) vaccination. AIM: To confirm immunogenicity of Diphtheria Tetanus acellular Pertussis (DTaP) vaccines administered SQ to individuals <6 years old with haemophilia. METHODS: We performed a retrospective and prospective pilot study of tetanus and diphtheria antibody titres among patients evaluated at our Haemophilia Treatment Centre between 2015-2016...
November 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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