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haemophilia A review

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https://www.readbyqxmd.com/read/28332238/outcome-measures-for-adult-and-pediatric-hemophilia-patients-with-inhibitors
#1
REVIEW
Cedric Hermans, Günter Auerswald, Gary Benson, Gerry Dolan, Anne Duffy, Victor Jiménez-Yuste, Rolf Ljung, Massimo Morfini, Thierry Lambert, Mehdi Osooli, Silva Zupančić Šalek
Recent advancements in almost all aspects of hemophilia treatment have vastly improved patient care and management, and new and emerging treatments hold the promise of further progress. However, there remains a scarcity of data on long-term outcomes in hemophilia, particularly among those patients with inhibitors, for whom no validated outcome assessment tools are currently available. At the 15(th) Zürich Haemophilia Forum, an expert panel reviewed the most important outcome measures in inhibitor patients and considered the challenges associated with assessing outcomes in this population...
March 22, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28306191/biomechanical-markers-and-theoretical-concepts-related-to-haemophilic-ankle-and-subtalar-joint-arthropathy-introducing-the-term-haemophilic-tarsal-pan-arthropathy
#2
REVIEW
S Lobet, A McCarthy, C Hermans, K Peerlinck, G A Matricali, F Staes, K Deschamps
BACKGROUND: Although regular factor replacement can reduce the incidence of joint bleeds and slow down the development of haemophilic arthropathy, the ankle joint remains particularly vulnerable even in children with haemophilia on primary or secondary prophylaxis and is now the primary joint affected. The heterogeneity in the pathoaetiology of haemophilic ankle arthropathy means that the functional consequences of early stage of ankle arthropathy are difficult to define as early morphological and structural changes can be observed in clinically asymptomatic ankles...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28302350/primary-prophylaxis-in-haemophilia-care-guideline-update-2016
#3
Kathelijn Fischer, Rolf Ljung
This paper reviews the current status on recommendations or guidelines for primary prophylaxis based on recent published papers from organizations or group of experts as well as some original key papers. A rather uniform view exists that prophylaxis should be initiated at an early age before or after no more than a single joint bleed and, if possible, preferably be continued for life. The dose and dose frequency of prophylaxis is dependent on the goal of treatment, bleeding phenotype, compliance, venous access and economic resources in the health care system and should be tailored individually based on clinical outcome and pharmacokinetics...
February 17, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28289747/physiotherapy-treatments-in-musculoskeletal-pathologies-associated-with-haemophilia-a-literature-review
#4
Jesús Guodemar-Pérez, Montserrat Ruiz-López, Elena Rodríguez-López, Pablo García-Fernández, Juan Pablo Hervás-Pérez
The aim of this study is to offer physiotherapists a synthesis of the main therapeutic tools available for the treatment of musculoskeletal pathologies in patients with haemophilia, according to the scientific literature. Although bleeds are recognised as no longer being a cause of death for people with haemophilia, the accompanying musculoskeletal injuries now represent the main problem associated with this disorder. There is a lack of clear guidelines to date regarding the physiotherapy treatment of these disorders...
March 14, 2017: Hämostaseologie
https://www.readbyqxmd.com/read/28118963/acquired-haemophilia-epidemiology-clinical-presentation-diagnosis-and-treatment
#5
REVIEW
Maria Eva Mingot-Castellano, Ramiro Núñez, Francisco Javier Rodríguez-Martorell
The development of circulating autoantibodies able to inhibit some coagulation proteins induces severe or even life-threatening bleeding. This disorder is called acquired haemophilia. This is a rare disease, although its impact may be underestimated because of the lack of records, the lack of knowledge by many specialists, the complexity of the laboratory diagnosis and, finally, because of the fulminant clinical presentation that often precludes diagnosis. Several studies established that mortality ranges between 9 and 33%...
April 7, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28111847/making-economic-evaluations-more-helpful-for-treatment-choices-in-haemophilia
#6
REVIEW
M Drummond, N Houwing, U Slothuus, P Giangrande
AIM: Poorly conducted economic evaluations have the potential to mislead both clinicians, leading to inappropriate treatment choices, and payers who must decide on the reimbursement of treatment costs. This paper reviews the methods used in economic evaluations in haemophilia and proposes standards for conducting and reporting such evaluations in the future. METHODS: A systematic review of economic evaluations in haemophilia published since 2008 was conducted. The reporting and methods of the studies were assessed using the recently published Consolidated Health Economic Evaluation Reporting Guidelines (CHEERS) checklist...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28101938/the-second-team-haemophilia-education-meeting-2016-frankfurt-germany
#7
Erik Berntorp, Yesim Dargaud, Daniel Hart, Sébastien Lobet, Maria Elisa Mancuso, Roseline d'Oiron, David Perry, Debra Pollard, Marijke van den Berg, Jan Blatný, Hervé Chambost, Andrea S Doria, Pål André Holme, Radoslaw Kaczmarek, Lorenzo Mantovani, Paul McLaughlin, Lochana Nanayakkara, Pia Petrini, Thomas Sannié, Edward Laane, Raquel Maia, Athina Dettoraki, Anna Farrell, Susan Halimeh, Sayma Raza, Stephanie Taylor
The first Team Haemophilia Education (THE) Meeting was held on 7-8 May 2015 in Amsterdam, The Netherlands. It aimed to promote the optimal care of patients with haemophilia through education of the multidisciplinary treatment team. This was achieved by reviewing the latest developments in haemophilia management, considering how these can be implemented in the clinic to improve patient care and providing a platform for networking and debate for all haemophilia treatment team members. The second THE Meeting was held on 19-20 May in Frankfurt, Germany, and participants included doctors, nurses, physiotherapists, patient representatives and data management staff from 20 different countries...
January 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/27996087/gene-therapy-for-haemophilia
#8
REVIEW
Akshay Sharma, Manu Easow Mathew, Vasumathi Sriganesh, Ulrike M Reiss
BACKGROUND: Haemophilia is a genetic disorder characterized by spontaneous or provoked, often uncontrolled, bleeding into joints, muscles and other soft tissues. Current methods of treatment are expensive, challenging and involve regular administration of clotting factors. Gene therapy has recently been prompted as a curative treatment modality. This is an update of a published Cochrane Review. OBJECTIVES: To evaluate the safety and efficacy of gene therapy for treating people with haemophilia A or B...
December 20, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27992070/exercise-for-haemophilia
#9
REVIEW
Karen Strike, Kathy Mulder, Rojer Michael
BACKGROUND: Haemophilia is a bleeding disorder associated with haemorrhaging into joints and muscles. Exercise is often used to aid recovery after bleeds, and to improve joint function in the presence of arthropathy. OBJECTIVES: Our objective was to systematically review the available evidence on the safety and effectiveness of exercise for people with haemophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register and electronic databases PubMed, OVID-Embase, and CINAHL...
December 19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27988873/coagulation-factor-ix-recombinant-albumin-fusion-protein-albutrepenonacog-alfa-idelvion-%C3%A2-a-review-of-its-use-in-haemophilia-b
#10
Katherine A Lyseng-Williamson
Albutrepenonacog alfa (Idelvion(®)), a fusion protein that genetically fuses recombinant factor IX (rFIX) with recombinant human albumin (rAlbumin), is indicated in the treatment of haemophilia B. This narrative review discusses the pharmacological properties and clinical data related to the use of this novel fusion protein, hereafter referred to as rIX-FP. The fusion of rFIX to rAlbumin prolongs the elimination half-life of rIX-FP in the circulation, allowing routine prophylaxis to be administered once every 7-14 days...
December 17, 2016: Drugs
https://www.readbyqxmd.com/read/27971804/review-of-national-health-technology-assessment-reports-on-treatment-of-severe-haemophilia-a
#11
I Odnoletkova
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27971789/cost-effectiveness-of-prophylaxis-compared-to-on-demand-treatment-in-severe-haemophilia-a-systematic-review
#12
I Odnoletkova
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27971716/the-mothif-study-costs-of-clotting-factors-in-haemophilia-a-and-b-a-review-from-the-berhlingo-database-in-seven-comprehensive-health-care-centres-in-france
#13
V Horvais, M Pennetier, G Chapelle, A Athouel, S Bauer, V Cogulet, C Lebert, M Clerc, I Vincent, Y Poirier, N Le Gall, V Pacaud, S Daubie, S Orhon-Ménard, M Gaumé, P Jaccard, M Haudot-Lépine, J Teil, J Huaut-Quentel, B Lassère, M Kuzzay, M Roy, A Gaudy, C Baubri, A Courtois, I Baudin Jacquemin-Aubry, N Ripoll, P Chauvet, B Pan-Petesch, L Ardillon, B Guillet, P Beurrier, F Pineau-Vincent, L Macchi, L Ferrand, M Trossaërt, Berhlingo Group
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27966004/management-of-cardiovascular-disease-in-aging-persons-with-haemophilia
#14
Roger E G Schutgens, Michiel Voskuil, Evelien P Mauser-Bunschoten
With the aging of the haemophilia population, age related comorbidities become more and more a medical issue. Managing haemophilia patients with cardiovascular disease is a difficult task for many haemophilia-treating physicians. Over the years, insights on prevalence, risk factors and management of cardiovascular disease in haemophilia have improved substantially. It is now recognised that many risk factors, such as hypertension and overweight, occur quite frequently in patients with haemophilia. Several new insights in anticoagulation management of atrial fibrillation and coronary ischaemia in haemophilia have been suggested...
December 14, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27862662/prevalence-and-severity-by-age-and-other-clinical-correlates-of-haemophilic-arthropathy-of-the-elbow-knee-and-ankle-among-taiwanese-patients-with-haemophilia
#15
C-Y Chang, T-Y Li, S-N Cheng, R-Y Pan, H-J Wang, S-Y Lin, Y-C Chen
INTRODUCTION & AIMS: Haemophilic arthropathy (HA) is a major complication in patients with haemophilia (PWH), but the study of age-specific prevalence and severity of HA is very limited in Asian countries. MATERIALS & METHODS: This study retrospectively reviewed 146 severe- and moderate-type Taiwanese PWH aged 4-73 years, with roentgenograms of elbows, knees and ankles and calculated Pettersson scores. RESULTS: The prevalence of HA, mean number of HAs per patient and mean Pettersson scores of all the joints were 42...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27824212/regulatory-t-cells-and-their-potential-for-tolerance-induction-in-haemophilia-a-patients
#16
A Schmidt, C Königs
FVIII inhibitors still are the major concern in treatment of haemophilia A patients by FVIII replacement therapy. Immune tolerance induction to reverse inhibitor formation fails in about 30% of treated patients. These patients face increased morbidity and mortality producing a need for new therapy strategies in the treatment of FVIII inhibitor-positive patients. Regulatory T cells are important modulators of the immune response and are also involved in the immune response to FVIII in haemophilia A patients...
November 8, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27790841/outcome-measures-in-european-patients-with-haemophilia-survey-of-implementation-in-routine-clinical-practice-perception-of-relevance-and-recommendations-by-european-treaters-in-the-ehtsb
#17
C Hermans, R Klamroth, M Richards, P de Moerloose, R P Garrido
INTRODUCTION: This study was conducted to evaluate the current implementation of outcome measures in routine clinical haemophilia practice and to explore and appreciate the perception of the relevance of such measures by treaters. METHODS: A survey was completed by 19 of the 26 physicians involved in the European Haemophilia Therapy Strategy Board (EHTSB). Employing an extensive inventory of outcome measures used in patients with haemophilia, information was collected about the frequency of data collection and the subjective appreciation of their importance during clinic review...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27770665/current-view-and-outcome-of-iti-therapy-a-change-over-time
#18
K Holstein, A Batorova, M Carvalho, K Fijnvandraat, P Holme, K Kavakli, T Lambert, A Rocino, V Jiménez-Yuste, J Astermark
INTRODUCTION: Inhibitor development in people with haemophilia is a serious complication that may require intensive and costly interventions. The goal of inhibitor management should be permanent inhibitor eradication through immune tolerance induction (ITI), but well-designed studies are lacking and the management of patients is therefore defined by the experience and views of the clinician. OBJECTIVES: To explore the current clinical practice and outcome of ITI therapy in Europe and how this may have changed over the last decade, as well as to provide consensus recommendations to guide clinicians in their clinical practice...
December 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27633342/choosing-outcome-assessment-tools-in-haemophilia-care-and-research-a-multidisciplinary-perspective
#19
REVIEW
K Fischer, P Poonnoose, A L Dunn, P Babyn, M J Manco-Johnson, J A David, J van der Net, B Feldman, K Berger, M Carcao, P de Kleijn, M Silva, P Hilliard, A Doria, A Srivastava, V Blanchette
INTRODUCTION: The implementation of early long-term, regular clotting factor concentrate (CFC) replacement therapy ('prophylaxis') has made it possible to offer boys with haemophilia a near normal life. Many different regimens have reported favourable results, but the optimum treatment regimens have not been established and the cost of prophylaxis is very high. Both for optimizing treatment and reimbursement issues, there is a need to provide objective evidence of both short- and long-term results and benefits of prophylactic regimens...
January 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27599770/evidence-for-and-cost-effectiveness-of-physiotherapy-in-haemophilia-a-dutch-perspective
#20
P de Kleijn, E P Mauser-Bunschoten, K Fischer, C Smit, H Holtslag, C Veenhof
INTRODUCTION: Musculoskeletal impact of haemophilia justifies physiotherapy throughout life. Recently the Dutch Health Care Institute constrained their 'list of chronic conditions', and withdrew financial coverage of physiotherapy for elderly persons with haemophilia (PWH). This decision was based on lack of scientific evidence and not being in accordance with 'state of science and practice'. METHODS: In general, evidence regarding physiotherapy is limited, and especially in rare diseases like haemophilia...
November 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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