journal
MENU ▼
Read by QxMD icon Read
search

Haemophilia: the Official Journal of the World Federation of Hemophilia

journal
https://www.readbyqxmd.com/read/28449213/femoral-head-fracture-in-a-haemophilic-arthropathy-patient-a-case-report-from-china
#1
LETTER
S Zhang, H Qu, H Jin, X Jiang, P Tong
No abstract text is available yet for this article.
April 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28440032/discrepancies-between-the-one-stage-clotting-assay-and-the-chromogenic-assay-in-haemophilia-b
#2
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
#3
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
#4
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
#5
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/28439934/an-international-prophylaxis-study-group-ipsg-survey-of-prophylaxis-in-inhibitor-positive-children-adults-with-severe-haemophilia
#6
LETTER
M D Carcao, L Avila, C Leissinger, V S Blanchette, L Aledort
No abstract text is available yet for this article.
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429882/reply-to-the-letter-by-iorio
#7
LETTER
P M Mannucci, F Peyvandi, F R Rosendaal
No abstract text is available yet for this article.
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429878/a-systematic-review-of-ultrasound-imaging-as-a-tool-for-evaluating-haemophilic-arthropathy-in-children-and-adults
#8
REVIEW
C C Ligocki, A Abadeh, K C Wang, T Adams-Webber, V S Blanchette, A S Doria
The purpose of this study was to semi-quantitatively assess the evidence on the value of ultrasound (US) for assessment of haemophilic arthropathy (HA) in children and adults based on the following questions: (1) Does early diagnosis of pathological findings, using available US techniques, impact the functional status of the joint? (2) Do current available US techniques have the ability to accurately detect pathological changes in target joints in haemophilic patients? (3) Does treatment (prophylaxis) improve US evidence of haemophilic arthropathy in children and adults? (4) Is there any association between various US scoring systems and other clinical/radiological constructs? Of the 6880 citations identified searching databases such as MEDLINE, Embase, CENTRAL and Web of Science, 20 articles investigating either the diagnostic accuracy of US and/or US scanning protocols and scoring systems for assessment of HA met the inclusion criteria for the study...
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429874/an-international-prophylaxis-study-group-ipsg-survey-of-prophylaxis-in-adults-with-severe-haemophilia
#9
LETTER
M D Carcao, L Avila, C Leissinger, V S Blanchette, L Aledort
No abstract text is available yet for this article.
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429867/health-related-quality-of-life-questionnaires-in-individuals-with-haemophilia-a-systematic-review-of-their-measurement-properties
#10
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
#11
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/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
#12
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/28401658/kreuth-iv-european-consensus-proposals-for-treatment-of-haemophilia-with-coagulation-factor-concentrates
#13
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/28393468/the-association-between-health-utility-and-joint-status-among-people-with-severe-haemophilia-a-findings-from-the-kappa-register
#14
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/28371032/insight-into-health-related-quality-of-life-of-young-children-with-haemophilia-b-treated-with-long-acting-nonacog-beta-pegol-recombinant-factor-ix
#15
LETTER
M Carcao, S Kearney, E Santagostino, J O O Oyesiku, N L Young, J Meunier, C S Hoxer, C Zhang, V S Blanchette
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/28370924/guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia-a-united-kingdom-haemophilia-centre-doctors-organisation-ukhcdo-guideline
#16
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/28370910/experience-of-immune-tolerance-in-a-carrier-of-severe-haemophilia-a-with-inhibitor-development-post-surgery
#17
LETTER
R C Trickey, C Percy, P V Jenkins, R Harris, C Loran, P W Collins
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
#18
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
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
#19
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/28345289/severe-bleeding-diatheses-in-an-elderly-patient-with-combined-type-autoantibody-against-factor-xiii-a-subunit-novel-approach-to-the-diagnosis-and-classification-of-anti-factor-xiii-antibodies
#20
M Kun, N Szuber, É Katona, K Pénzes, A Bonnefoy, B Bécsi, F Erdődi, G E Rivard, L Muszbek
INTRODUCTION: Acquired factor XIII (FXIII) deficiency due to autoantibody is a rare, severe bleeding diathesis. Its laboratory diagnosis and classification represents a difficult task. AIM: Introduction of novel approaches into the diagnosis and characterization of anti-FXIII autoantibody and demonstration of their use in the diagnosis of a patient with autoimmune FXIII deficiency. METHODS: Factor XIII activity, FXIII antigen levels and the titre of anti-FXIII-A antibody were monitored throughout the course of the disease...
March 26, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
journal
journal
32138
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"