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https://www.readbyqxmd.com/read/28440032/discrepancies-between-the-one-stage-clotting-assay-and-the-chromogenic-assay-in-haemophilia-b
#1
K Kihlberg, K Strandberg, S Rosén, R Ljung, J Astermark
INTRODUCTION: Assay discrepancy in factor VIII activity between the one-stage and the chromogenic assays has been described in approximately one third of patients with non-severe haemophilia A. Whether assay discrepancy may also occur in patients with haemophilia B remains unknown. AIM: This study compared the results from the one-stage and the chromogenic assays in patients with haemophilia B. METHODS: Plasma samples from patients with haemophilia B attending the haemophilia centre in Malmö, Sweden, were collected after a wash-out period of more than 7 days and analysed with both assays...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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
#2
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/28440004/safety-and-effectiveness-of-room-temperature-stable-recombinant-factor-viia-in-patients-with-haemophilia-a-or-b-and-inhibitors-results-of-a-multinational-prospective-observational-study
#3
K Kavakli, F Demartis, M Karimi, P Eshghi, D Neme, H Chambost, L Sommer, M Zak, G Benson
INTRODUCTION: A room temperature stable formulation of recombinant activated factor VII (NovoSeven(®) ), allowing convenient storage and therefore improved treatment access, has been developed. Bioequivalence to the previous NovoSeven(®) was demonstrated in healthy humans, leading to European approval (2008). Although no confirmed cases of neutralising antibodies to rFVIIa in patients with haemophilia A or B have been observed with the original formulation, changes in formulation or storage condition may alter immunogenicity...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28439941/serological-biomarkers-detect-active-joint-destruction-and-inflammation-in-patients-with-haemophilic-arthropathy
#4
B Hua, E H N Olsen, S Sun, C N Gudme, L Wang, B Vandahl, K Roepstorff, M Kjelgaard-Hansen, B B Sørensen, Y Zhao, M A Karsdal, T Manon-Jensen
INTRODUCTION: Progressive arthropathy caused by recurrent joint bleeds is a severe complication in haemophilia. AIM: We investigated whether biomarkers of cartilage and bone degradation, and inflammation were altered in haemophilia patients and whether these biomarkers could identify haemophilia patients with arthropathy. METHODS: Serum from 35 haemophilia patients with varying degrees of arthropathy and 43 age- and gender-matched control subjects were analysed...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28438758/diabetes-mellitus-and-acquired-haemophilia-new-association
#5
Carlos Tavares Bello, Yuliya Vasylenko, José Esteves, Carlos Augusto Vasconcelos
Diabetes mellitus encompasses a group of highly prevalent carbohydrate metabolic disorders with an increasing incidence. Some subtypes are thought to be associated with other immune-mediated diseases. Acquired haemophilia on the other hand is a quite rare autoimmune disease that is thought to be secondary to the emergence of inhibiting anticoagulation factor VIII antibodies (inhibitors) in patients with previously normal haemostatic function. More recently, numerous different diseases have been associated with acquired haemophilia namely immune-mediated diseases, drugs and solid and haematologic neoplasms...
April 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28429867/health-related-quality-of-life-questionnaires-in-individuals-with-haemophilia-a-systematic-review-of-their-measurement-properties
#6
REVIEW
P F Limperg, C B Terwee, N L Young, V E Price, S C Gouw, M Peters, M A Grootenhuis, V Blanchette, L Haverman
BACKGROUND: The evaluation of health related quality of life (HRQOL) is essential for a full assessment of the influence of an illness on patients' lives. The aim of this paper is to critically appraise and compare the measurement properties of HRQOL questionnaires studied in haemophilia. METHODS: Bibliographic databases (Embase, Medline, Cinahl and PsycInfo) were searched for articles evaluating measurement properties of HRQOL questionnaires in haemophilia. Articles were excluded that did not report HRQOL measurement properties, or when <50% of the study population had haemophilia...
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429865/differential-effects-of-bleeds-on-the-development-of-arthropathy-basic-and-applied-issues
#7
REVIEW
L F D van Vulpen, S C Mastbergen, F P J G Lafeber, R E G Schutgens
Haemophilia is characterized by a spontaneous bleeding tendency, affecting mainly the synovial joints. Recurrent joint bleeds induce a cascade of inflammatory as well as degenerative processes injuring synovium, cartilage and bone. These processes affect each other and may occur in parallel and/or sequentially. Clinically, the effects of joint bleeds are heterogeneous. A marked variability in joint damage is observed in patients with a similar bleeding history. Also late stage effects differ with some patients developing chronic synovitis, and others suffering from osteochondral degeneration called haemophilic arthropathy...
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28424853/long-term-results-of-ankle-arthrodesis-in-children-and-adolescents-with-haemophilia
#8
Nicolas de l'Escalopier, Alina Badina, Jean Paul Padovani, Annie Harroche, Laurent Frenzel, Philippe Wicart, Christophe Glorion, Chantal Rothschild
INTRODUCTION: Ankle arthrodesis in patients with haemophilia is a joint-non-preserving treatment option often resulting in substantial pain relief and termination of haemarthrosis by osseous consolidating of the joint. However, limited data are available regarding ankle arthrodesis in young patients. AIM: The aim of this study was to evaluate the long-term clinical and radiographic results with a minimum follow-up of ten years in children with haemophilia who underwent ankle arthrodesis...
April 20, 2017: International Orthopaedics
https://www.readbyqxmd.com/read/28420167/treatment-and-prevention-of-bleeds-in-haemophilia-patients-with-inhibitors-to-factor-viii-ix
#9
REVIEW
Angiola Rocino, Massimo Franchini, Antonio Coppola
The development of alloantibodies neutralising therapeutically administered factor (F) VIII/IX (inhibitors) is currently the most severe complication of the treatment of haemophilia. When persistent and at a high titre, inhibitors preclude the standard replacement treatment with FVIII/FIX concentrates, making patients' management challenging. Indeed, the efficacy of bypassing agents, i.e., activated prothrombin complex concentrates (aPCC) and recombinant activated factor VII (rFVIIa), needed to overcome the haemostatic interference of the inhibitor, is not comparable to that of factor concentrates...
April 17, 2017: Journal of Clinical Medicine
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
#10
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/28419629/motivational-and-behavioural-models-of-change-a-longitudinal-analysis-of-change-among-men-with-chronic-haemophilia-related-joint-pain
#11
J Elander, C Richardson, J Morris, G Robinson, M B Schofield
BACKGROUND: Motivational and behavioural models of adjustment to chronic pain make different predictions about change processes, which can be tested in longitudinal analyses. METHODS: We examined changes in motivation, coping and acceptance among 78 men with chronic haemophilia-related joint pain. Using cross-lagged regression analyses of changes from baseline to 6 months as predictors of changes from 6 to 12 months, with supplementary structural equation modelling, we tested two models in which motivational changes influence behavioural changes, and one in which behavioural changes influence motivational changes...
April 17, 2017: European Journal of Pain: EJP
https://www.readbyqxmd.com/read/28401658/kreuth-iv-european-consensus-proposals-for-treatment-of-haemophilia-with-coagulation-factor-concentrates
#12
P L F Giangrande, F Peyvandi, B O'Mahony, M-E Behr-Gross, A Hilger, W Schramm, P M Mannucci
INTRODUCTION: This report summarizes recommendations relating to haemophilia therapy arising from discussions among experts from 36 European countries during the 'Kreuth IV' meeting in May 2016. AIM: The objective of the meeting was for experts in the field of haemophilia from across Europe to draft resolutions regarding current issues relating to the treatment of haemophilia. RESULTS: Hospitals providing clinical care for people with haemophilia and related disorders are strongly recommended to seek formal designation as either European Haemophilia Treatment Centres (EHTC) or European Haemophilia Comprehensive Care Centres (EHCCC)...
April 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28397753/hip-arthropathy-in-haemophilia
#13
Christian Carulli, Anna Rosa Rizzo, Massimo Innocenti
Hip arthropathy in haemophilic patients is disabling for hip and other common target joints. Even if bleedings in the hip are not frequent, femoroacetabular alterations may affect the functional ability of patients at a very young age. A haematologic prophylaxis combined with an adequate lifestyle and regular and low-traumatic physical activity are the keys to preventing such arthropathy. In the early stages of arthropathy, anti-inflammatory drugs and physical therapy may be sufficient to limit its progression...
April 8, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28394285/diagnosis-and-treatment-of-von-willebrand-disease-and-rare-bleeding-disorders
#14
REVIEW
Giancarlo Castaman, Silvia Linari
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF...
April 10, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28393961/wishes-and-worries-of-haemophilia-patients-a-patient-survey-from-bavaria
#15
Herbert Lechner, Anja Schleiermacher, Karin Berger, Dorothee Schopohl, Wolfgang Schramm
Haemophilia care in Germany has achieved a high level and enables the majority of patients to lead a largely normal life. The Bluter Betreuung Bayern e.V. (BBB) aims to improve health care and support for haemophilia patients. A questionnaire has been developed by BBB representatives to evaluate unmet medical needs from the patient perspective. It was sent to 290 haemophilia patients and/or their parents in Bavaria in November 2015. The response rate was 51.4 %: 66 children aged < 15 years (66.7 % severe), 30 patients 15-24 years (66...
April 10, 2017: Hämostaseologie
https://www.readbyqxmd.com/read/28393468/the-association-between-health-utility-and-joint-status-among-people-with-severe-haemophilia-a-findings-from-the-kappa-register
#16
M Osooli, K Steen Carlsson, F Baghaei, M Holmström, S Rauchensteiner, P A Holme, L Hvitfeldt, J Astermark, E Berntorp
INTRODUCTION: People with severe haemophilia A have reportedly impaired health related quality of life (utility) mainly due to recurrent bleeding, arthropathy and treatment burden. AIM: To estimate utilities and evaluate their potential correlates - most importantly the joint status - among people with severe haemophilia A. METHODS: In this cross-sectional study, eligible participants had severe haemophilia A, were aged ≥15, negative for factor VIII inhibitor and included in the KAPPA register of Denmark, Norway and Sweden...
April 10, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28387451/large-scale-studies-assessing-anti-factor-viii-antibody-development-in-previously-untreated-haemophilia-a-what-has-been-learned-what-to-believe-and-how-to-learn-more
#17
REVIEW
Alfonso Iorio, Kathelijn Fischer, Michael Makris
Minimizing the risk of inhibitor development by acting on modifiable risk factors remains a sensible goal for treatment optimization in haemophilia A. By critically appraising published studies assessing inhibitor development, this review addresses the role of studies in previously untreated patients (PUPs) for establishing the immunogenicity of new concentrates, suggest novel research design to be adopted in future studies and discuss clinical practice implications of the reported differential immunogenicity of Kogenate Bayer and Advate factor VIII concentrates...
April 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28384182/global-measurement-of-coagulation-in-plasma-from-normal-and-haemophilia-dogs-using-a-novel-modified-thrombin-generation-test-demonstrated-in-vitro-and-ex-vivo
#18
Daniel Elenius Madsen, Timothy C Nichols, Elizabeth P Merricks, Emily K Waters, Bo Wiinberg
INTRODUCTION: Canine models of severe haemophilia resemble their human equivalents both regarding clinical bleeding phenotype and response to treatment. Therefore pre-clinical studies in haemophilia dogs have allowed researchers to make valuable translational predictions regarding the potency and efficacy of new anti-haemophilia drugs (AHDs) in humans. To refine in vivo experiments and reduce number of animals, such translational studies are ideally preceded by in vitro prediction of compound efficacy using a plasma based global coagulation method...
2017: PloS One
https://www.readbyqxmd.com/read/28370924/guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia-a-united-kingdom-haemophilia-centre-doctors-organisation-ukhcdo-guideline
#19
REVIEW
J Hanley, A McKernan, M D Creagh, S Classey, P McLaughlin, N Goddard, P J Briggs, S Frostick, P Giangrande, J Wilde, J Thachil, P Chowdary
No abstract text is available yet for this article.
March 30, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28370896/haemophilia-patients-unmet-needs-and-their-expectations-of-the-new-extended-half-life-factor-concentrates
#20
S von Mackensen, W Kalnins, J Krucker, J Weiss, W Miesbach, M Albisetti, I Pabinger, J Oldenburg
INTRODUCTION: National Member Organisations (NMO) of persons with haemophilia (PWH) from the DACH Region (D = Germany, A = Austria, CH = Switzerland) were interested to better understand PWH's expectations and concerns of extended half-life (EHL) factor concentrates (FC) before availability in these countries. METHODS: Based on an expert meeting and focus groups conducted across Germany a survey for haemophilia patients and their parents was developed and was sent out to 2,644 PWH...
March 30, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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