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https://www.readbyqxmd.com/read/28440005/comparing-the-burden-of-illness-of-haemophilia-between-resource-constrained-and-unconstrained-countries-the-s%C3%A3-o-paulo-toronto-hemophilia-study
#1
J D A Carneiro, V Blanchette, M C Ozelo, S V Antunes, P R Villaca, N L Young, D Castro, L R Brandão, M Carcao, A Abad, B M Feldman
INTRODUCTION: Although the regular replacement of clotting factor concentrates (prophylaxis) has been well established as the standard of care for severe haemophilia, the high cost of factor concentrates has limited access to prophylaxis in countries with under-developed or developing economies. AIMS: We studied the health gap that could be addressed by providing unlimited access to clotting factor concentrates with implementation of long-term prophylaxis initiated from an early age in life...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28419637/self-reported-prevalence-description-and-management-of-pain-in-adults-with-haemophilia-methods-demographics-and-results-from-the-pain-functional-impairment-and-quality-of-life-p-fiq-study
#2
M Witkop, A Neff, T W Buckner, M Wang, K Batt, C M Kessler, D Quon, L Boggio, M Recht, K Baumann, R Z Gut, D L Cooper, C L Kempton
INTRODUCTION: Haemophilia is characterized by frequent haemarthrosis, leading to acute/chronic joint pain. AIM: To assess self-reported prevalence, description and management of pain in adult males with mild-to-severe haemophilia and history of joint pain/bleeding. METHODS: Participants completed a pain survey and five patient-reported outcome instruments assessing pain, functional impairment and health-related quality of life (HRQoL). RESULTS: Of 381 participants enrolled, median age was 34 years; 77% had haemophilia A, 71% had severe disease and 65% were overweight/obese...
April 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28345299/a-prospective-study-of-health-related-quality-of-life-of-boys-with-severe-haemophilia-a-in-china-comparing-on-demand-to-prophylaxis-treatment
#3
R Wu, J Sun, J Xiao, Y Liu, F Xue, H Wang, L Tang, Y Zhao, K Li, R Yang, Y Hu, K-H Luke, M-C Poon, V S Blanchette, K Usuba, N L Young
INTRODUCTION: Treatment for boys with haemophilia in China is rapidly improving; however, comprehensive outcomes have not been examined prospectively. AIM: The aim of this study was to evaluate the effect of short-term full-dose prophylaxis compared to on-demand treatment, on the Health-Related Quality of Life (HR-QoL) of boys with severe haemophilia A (HA) in China. METHODS: Boys with severe HA (FVIII<1%) completed 3 months of on-demand treatment and 3 months of full-dose prophylaxis (25 FVIII IU per kg 3x per week)...
March 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28332238/outcome-measures-for-adult-and-pediatric-hemophilia-patients-with-inhibitors
#4
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/28321962/psychosocial-care-for-children-with-haemophilia-and-their-parents-in-the-netherlands
#5
REVIEW
P F Limperg, L Haverman, M Beijlevelt, M van der Pot, G Zaal, W A de Boer, K Fijnvandraat, M Peters, M A Grootenhuis
INTRODUCTION: Children growing up with haemophilia are at greater risk for psychosocial problems than their healthy peers. Providing psychosocial care to children with haemophilia and their families is indispensable, since psychosocial factors can have a significant impact on health and health-related quality of life (HRQOL). AIMS: Our aim was to give a description of psychosocial care provided by the multidisciplinary team of the Hemophilia Comprehensive Care Centre (HCCC) at the Emma Children's Hospital in Amsterdam, the Netherlands...
March 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28187737/evaluation-of-a-web-based-registry-of-inherited-bleeding-disorders-a-descriptive-study-of-the-brazilian-experience-with-hemovidaweb-coagulopatias
#6
Suely Meireles Rezende, Silvia Helena Lacerda Rodrigues, Kelly Neves Pinheiro Brito, Diego Lima Quintino da Silva, Marcos Lázaro Santo, Bárbara de Jesus Simões, Guilherme Genovez, Helder Teixeira Melo, João Paulo Baccara Araújo, Danila Augusta Accioly Varella Barca
BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27992070/exercise-for-haemophilia
#7
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/27928881/measuring-the-quality-of-haemophilia-care-across-different-settings-a-set-of-performance-indicators-derived-from-demographics-data
#8
MULTICENTER STUDY
A Iorio, J S Stonebraker, M Brooker, J M Soucie
BACKGROUND: Haemophilia is a rare disease for which quality of care varies around the world. We propose data-driven indicators as surrogate measures for the provision of haemophilia care across countries and over time. MATERIALS AND METHODS: The guiding criteria for selection of possible indicators were ease of calculation and direct applicability to a wide range of countries with basic data collection capacities. General population epidemiological data and haemophilia A population data from the World Federation of Hemophilia (WFH) Annual Global Survey (AGS) for the years 2013 and 2010 in a sample of 10 countries were used for this pilot exercise...
January 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27914734/applying-a-deliberation-model-to-the-analysis-of-consultations-in-haemophilia-implications-for-doctor-patient-communication
#9
Giulia Lamiani, Sarah Bigi, Maria Elisa Mancuso, Antonio Coppola, Elena Vegni
OBJECTIVE: Literature highlights the importance of communication in order to achieve patient's adherence. However, the specific dialogical components likely to favor patient adherence are not clear. In this study, the deliberation dialogue model was applied as an ideal model of optimal deliberation to real physician-patient consultations in the field of hemophilia in order to identify misalignments with the model and possible improvements in physician-patient communication. METHODS: By applying the deliberation model, we analyzed a corpus of 30 check-up consultations in hemophilia...
April 2017: Patient Education and Counseling
https://www.readbyqxmd.com/read/27813214/complications-of-haemophilia-in-babies-first-two-years-of-life-a-report-from-the-centers-for-disease-control-and-prevention-universal-data-collection-system
#10
R Kulkarni, R J Presley, J M Lusher, A D Shapiro, J C Gill, M Manco-Johnson, M A Koerper, T C Abshire, D DiMichele, W K Hoots, P Mathew, D J Nugent, S Geraghty, B L Evatt, J M Soucie
AIM: To describe the prevalence and complications in babies ≤2 years with haemophilia. METHODS: We used a standardized collection tool to obtain consented data on eligible babies aged ≤2 years with haemophilia enrolled in the Centers for Disease Control and Prevention Universal Data Collection System surveillance project at US Hemophilia Treatment Centers (HTCs). RESULTS: Of 547 babies, 82% had haemophilia A, and 70% were diagnosed within one month of birth...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27686244/physician-trust-and-depression-influence-adherence-to-factor-replacement-a-single-centre-cross-sectional-study
#11
D Q Tran, V Barry, A Antun, M Ribeiro, S Stein, C L Kempton
INTRODUCTION: Poor adherence to factor replacement therapy among patients with haemophilia can lead to joint bleeding and eventual disability. AIM: The aim of this study was to determine patient-related characteristics associated with adherence to factor replacement in adults with haemophilia. METHODS: Adults with haemophilia were recruited to participate in this cross-sectional study. Adherence was measured using either the Validated Hemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro or the VERITAS-PRN questionnaire...
January 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27661241/sporting-activities-and-quality-of-life-in-children-with-hemophilia-an-observational-study
#12
Ruben Cuesta-Barriuso, Ana Torres-Ortuño, Sofía Pérez-Alenda, Juan José Carrasco, Felipe Querol, Joaquín Nieto-Munuera
INTRODUCTION: Sports activities are part of multidisciplinary treatments in people with hemophilia. AIM: The objective of this study was to assess the incidence of sports activities in the quality of life as perceived by children with hemophilia. METHODS: A total of 53 children with hemophilia aged 7 to 13 years and 51 children without hemophilia were evaluated. The perception of quality of life, clinical variables, and the frequency of sports activities were registered...
2016: Pediatric Physical Therapy
https://www.readbyqxmd.com/read/27510890/predicting-the-outcomes-of-using-longer-acting-prophylactic-factor-viii-to-treat-people-with-severe-hemophilia-a-a-hypothetical-decision-analysis
#13
A H Miners, S Krishnan, K J Pasi
Essentials No randomized trials have compared long-acting factor VIII (FVIII) with currently used products. A comparison was undertaken using a decision model to predict FVIII use and number of bleeds. In the base case, longer acting FVIII reduced factor use by 17% while resulting in similar bleeds. The value of longer acting FVIII will be largely determined by existing regimens and unit price. Click to hear Prof. Makris's presentation on new treatments in hemophilia SUMMARY: Background Recently, factor VIII (FVIII) products with longer half-lives, such as recombinant FVIII Fc fusion protein (rFVIIIFc), have become available...
November 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27387850/rituximab-for-eradicating-inhibitors-in-people-with-acquired-haemophilia-a
#14
REVIEW
Yan Zeng, Ruiqing Zhou, Xin Duan, Dan Long
BACKGROUND: Acquired haemophilia A is a rare bleeding disorder caused by the development of specific autoantibodies against coagulation factor VIII. Rituximab may be an alternative approach to the treatment of acquired haemophilia by eradicating FVIII autoantibodies. OBJECTIVES: To assess and summarise the efficacy and adverse effects of rituximab for treating people with acquired haemophilia A. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's trials registers, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings...
2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27385495/correlating-clinical-and-radiological-assessment-of-joints-in-haemophilia-results-of-a-cross-sectional-study
#15
P M Poonnoose, P Hilliard, A S Doria, S N Keshava, S Gibikote, M L Kavitha, B M Feldman, V Blanchette, A Srivastava
OBJECTIVES: This study was undertaken to determine the correlation between the radiological changes in haemophilic arthropathy [X-ray, Ultrasound (US) and MRI] and clinical assessment as determined by the Hemophilia Joint Health Score (HJHS); and to document the US and MRI changes in joints that appear normal on plain X-ray and clinical evaluation. MATERIALS AND METHODS: Of 55 study joints (22 knees and 33 ankles) in 51 patients with haemophilia/von Willebrand disease, with a median age of 15 years (range: 5-17) were assessed using X-rays (Pettersson score) and clinical examination (HJHS) at two centres (Toronto, Canada; Vellore, India)...
November 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27348398/understanding-stakeholder-important-outcomes-and-perceptions-of-equity-acceptability-and-feasibility-of-a-care-model-for-haemophilia-management-in-the-us-a-qualitative-study
#16
S J Lane, N S Sholapur, C H T Yeung, A Iorio, N M Heddle, M Sholzberg, M Pai
INTRODUCTION: Care for persons with haemophilia (PWH) is most commonly delivered through the integrated care model used by Hemophilia Treatment Centers (HTCs). Although this model is widely accepted as the gold standard for the management of haemophilia; there is little evidence comparing different care models. AIM: We performed a qualitative study to gain insight into issues related to outcomes, acceptability, equity and feasibility of different care models operating in the US...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27348397/methodology-for-the-development-of-the-nhf-mcmaster-guideline-on-care-models-for-haemophilia-management
#17
M Pai, N Santesso, C H T Yeung, S J Lane, H J Schünemann, A Iorio
BACKGROUND: Rigorous and transparent methods are necessary to develop clinically relevant and evidence-based practice guidelines. We describe the development of the National Hemophilia Foundation-McMaster Guideline on Care Models for Haemophilia Management, which addresses best practices in haemophilia care delivery. METHODS: We assembled a Panel of persons with haemophilia (PWH), parents of PWH, clinical experts and guideline methodologists. Conflicts of interest were disclosed and managed throughout...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27116081/physical-activity-in-individuals-with-haemophilia-and-experience-with-recombinant-factor-viii-fc-fusion-protein-and-recombinant-factor-ix-fc-fusion-protein-for-the-treatment-of-active-patients-a-literature-review-and-case-reports
#18
REVIEW
Michael Wang, María Teresa Álvarez-Román, Pratima Chowdary, Doris V Quon, Kim Schafer
The World Federation of Hemophilia and the National Hemophilia Foundation encourage people with haemophilia (PWH) to participate in routine physical activity. The benefits of physical activity for PWH include improvements in joint, bone, and muscle health. Accordingly, a number of studies suggest that levels of physical activity among PWH are similar to those of their healthy peers, especially among individuals who began prophylaxis at an early age (≤3 years). Importantly, several studies found either no increased risk or only a transient increase in risk of bleeding with more intensive physical activity compared with less intensive physical activity...
October 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27075748/physical-exercise-pain-and-musculoskeletal-function-in-patients-with-haemophilia-a-systematic-review
#19
REVIEW
G S Schäfer, S Valderramas, A R Gomes, M B Budib, Á L P Wolff, A A T Ramos
INTRODUCTION: Exercise can provide numerous benefits to haemophilia patients, including bleeding reduction in muscles and joints. AIM: This systematic review (SR) aims to evaluate the effects of physical exercise on pain and the musculoskeletal function of patients with hemophilia. METHODS: Literature searches of Pubmed, Web of Science, PEDro, Cochrane, Clinical Trials SciELO and Lilacs were performed. The risks of bias were measured using the JADAD scale...
May 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27071049/a-web-site-to-improve-management-of-patients-with-inherited-bleeding-disorders-in-the-emergency-department-results-at-2-years
#20
MULTICENTER STUDY
Annarita Tagliaferri, Caterina Di Perna, Chiara Biasoli, Gianna Franca Rivolta, Gabriele Quintavalle, Gianfranco Cervellin, Marco Barozzi, Laura Benedettini, Corrado Pattacini
Treatment of patients with inherited bleeding disorders (PWIBD) in the emergency department (ED) is challenging. In 2010, a project was started involving all eight hemophilia centers (HC) and all 44 EDs of the Region of Emilia-Romagna (Italy) to improve emergency care for PWIBD. The project incorporates guidelines for emergency treatment, education for ED staff, and a dedicated Web site providing extensive information, proposing treatments, and sharing data with patients' electronic clinical records. A Web algorithm, accessible to PWIBD as well as ED and HC staff, suggests the first dose of concentrate for each type and severity of bleed or trauma...
July 2016: Seminars in Thrombosis and Hemostasis
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