keyword
MENU ▼
Read by QxMD icon Read
search

haemophilia A review

keyword
https://www.readbyqxmd.com/read/28118963/acquired-haemophilia-epidemiology-clinical-presentation-diagnosis-and-treatment
#1
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%...
January 21, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28111847/making-economic-evaluations-more-helpful-for-treatment-choices-in-haemophilia
#2
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...
January 22, 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
#3
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
#4
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...
20, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27992070/exercise-for-haemophilia
#5
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...
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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...
November 8, 2016: 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
#12
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
#13
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...
October 27, 2016: 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
#14
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
#15
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...
September 15, 2016: 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
#16
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
https://www.readbyqxmd.com/read/27577234/bay-81-8973-octocog-alfa-kovaltry-%C3%A2-a-review-in-haemophilia-a
#17
REVIEW
Gillian M Keating
BAY 81-8973 (octocog alfa; Kovaltry(®)) is an unmodified, full-length, recombinant factor VIII (FVIII) concentrate with the same amino acid sequence as Kogenate(®) FS, but produced with innovative manufacturing technologies. This narrative review discusses the clinical efficacy and tolerability of BAY 81-8973 in haemophilia A, as well as summarizing its pharmacological properties. Results of the LEOPOLD I, LEOPOLD II and LEOPOLD Kids trials demonstrated that routine prophylaxis with intravenous BAY 81-8973 was associated with a low annualized bleeding rate (ABR) in previously treated adult and paediatric patients with severe haemophilia A...
October 2016: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/27577042/spontaneous-multilevel-airway-haemorrhage-in-acquired-haemophilia-a
#18
Anna See, Siti Radhziah Sudirman, Xin Yong Huang
BACKGROUND: Acquired haemophilia A is caused by the development of an autoantibody to factor VIII in a person with previously normal haemostasis. The most common clinical presentation in hereditary haemophilia is intra-articular bleeding. In contrast, acquired haemophilia more commonly presents with skin, soft tissue and mucosal haemorrhages. METHODS: We present a case of a patient with idiopathic acquired haemophilia A, whose initial presentation was that of spontaneous submental and submandibular haematoma which rapidly progressed to involve multiple sites in the airway, ultimately necessitating an emergent tracheotomy...
August 30, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27562315/non-genetic-risk-factors-in-haemophilia-a-inhibitor-management-the-danger-theory-and-the-use-of-animal-models
#19
REVIEW
K M Lövgren, H Søndergaard, S Skov, B Wiinberg
In haemophilia A (HA) management, antidrug antibodies, or inhibitors, are a serious complication that renders factor VIII (FVIII) replacement therapy ineffective, increases morbidity and reduces quality of life for affected patients. Inhibitor development aetiology is multifactorial and covers both genetic and therapy related risk factors. Many therapy-related risk factors have proven difficult to confirm due to several confounding factors and the small study populations available. However, clinical studies indicate that e...
September 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27561958/functional-outcome-of-total-knee-arthroplasty-in-patients-with-haemophilia
#20
S M J Mortazavi, B Haghpanah, M M Ebrahiminasab, T Baghdadi, G Toogeh
INTRODUCTION: Haemophilic arthropathy (HA) is a debilitating complication of haemophilia which leads to TKA in severe cases. AIM: We conducted a prospective study of the outcome of TKA in our haemophilia cohort to define the outcomes in this population and increase the cost effectiveness of the procedure in our developing country. METHODS: We reviewed patients with haemophilia who underwent TKA between April 2010 and April 2014. Patients with at least 6 months of follow-up were included...
November 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
keyword
keyword
112093
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"