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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/29218759/impact-of-pain-and-functional-impairment-in-us-adults-with-haemophilia-patient-reported-outcomes-and-musculoskeletal-evaluation-in-the-pain-functional-impairment-and-quality-of-life-p-fiq-study
#1
C L Kempton, M Recht, A Neff, M Wang, T W Buckner, A Soni, D Quon, M Witkop, L Boggio, R Z Gut, D L Cooper
INTRODUCTION: Standardized and disease-specific patient-reported outcome (PRO) instruments assessing pain, functional impairment and health-related quality of life (HRQoL) in people with haemophilia (PWH) have been used in studies, but infrequently in comprehensive care settings for individual assessment or treatment planning. AIM: To assess the impact of pain and functional impairment on HRQoL in PWH. METHODS: P-FiQ enrolled 381 adult PWH with a history of joint pain/bleeding and included 5 PROs and a clinical joint evaluation (Hemophilia Joint Health Score v2...
December 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29205699/use-of-telehealth-in-the-delivery-of-comprehensive-care-for-patients-with-haemophilia-and-other-inherited-bleeding-disorders
#2
REVIEW
R Kulkarni
Advances in technology such as telemedicine (TM) have made access to cost-effective, quality health care feasible for remote patients. TM is especially well suited for patients with chronic disorders such as haemophilia and related haemostatic disorders that benefit not only from more frequent interaction with care providers at a specialized haemophilia treatment center but also from consultations with other specialists. Telehealth refers to a broader application of TM and includes non-clinical services such as education, provider training, administrative meetings etc...
December 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29194866/break-through-bleeding-in-relation-to-pharmacokinetics-of-factor-viii-in-paediatric-patients-with-severe-haemophilia-a
#3
X Cheng, P Li, Z Chen, N Zhang, Y Zhen, L Zhao, X Wang, R Wu
INTRODUCTION: As the pharmacokinetics (PK) of factor VIII (FVIII) is individualized in children with haemophilia A (HA), PK parameters may be indicators of patients' bleeding phenotype and instruction for their personalized replacement program. AIM: The aim of this study was to investigate the possible relationship between PK/FVIII level and bleeding frequency in Chinese paediatric patients with severe (HA). METHODS: A total of 24 patients were enrolled in Beijing Children's Hospital from February to October 2015, all of whom were given 50 IU/kg of FVIII concentrates after a 72-hours washout period...
December 1, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29194852/the-role-of-sclerostin-dickkopf-1-and-receptor-activator-of-nuclear-factor-kb-ligand-osteoprotegerin-signalling-pathways-in-the-development-of-osteoporosis-in-patients-with-haemophilia-a-and-b-a-cross-sectional-study
#4
P Anagnostis, S Vakalopoulou, D Christoulas, S A Paschou, A Papatheodorou, V Garipidou, P Kokkoris, E Terpos
AIM: Haemophilia A and B are associated with reduced bone mineral density (BMD). The aim of this study was to assess circulating sclerostin and dickkopf-1 (Dkk-1), (inhibitors of osteoblastic differentiation), as well as the receptor activator of nuclear factor kB ligand (RANKL)/osteoprotegerin (OPG) system (the major regulator of osteoclastogenesis), in patients with haemophilia (PWH), their possible correlations with clinical risk factors and the effect of ibandronate on these markers...
December 1, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29193440/mid-term-outcomes-and-complications-of-total-knee-arthroplasty-in-haemophilic-arthropathy-a-review-of-consecutive-131-knees-between-2006-and-2015-in-a-single-institute
#5
S J Song, J K Bae, C H Park, M C Yoo, D K Bae, K I Kim
INTRODUCTION: Although total knee arthroplasty (TKA) in haemophilic arthropathy can reduce severe joint pain and improve functional disability, it is technically demanding. AIM: To evaluate mid-term outcomes and complications of TKA in haemophilic arthropathy. METHODS: This study retrospectively reviewed 131 consecutive primary TKAs in a single institute. The mean age was 41.0 years old, and the mean follow-up period was 6.8 years. Clinical and radiographic results were evaluated...
November 29, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29178608/molecular-investigation-of-41-patients-affected-by-coagulation-factor-xi-deficiency
#6
LETTER
V Rimoldi, E M Paraboschi, M Menegatti, F Peyvandi, O Salomon, S Duga, R Asselta
No abstract text is available yet for this article.
November 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29178325/the-spectrum-of-bleeding-in-women-and-girls-with-haemophilia-b
#7
REVIEW
J Staber, S E Croteau, J Davis, E F Grabowski, P Kouides, R F Sidonio
Although hemophilia B affects 1 in 25,000 males there may be 3 female hemophilia B carriers per affected male. This clinical review highlights the unique challenges faced by hemophilia B carriers including the under-recognition of bleeding symptoms associated with and without FIX deficiency, discrepancies in correlation between genotype and bleeding phenotype and therapeutic considerations utilizing clinical vignettes of common scenarios.
November 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29178209/harold-ross-roberts-md-1930-2017
#8
D M Monroe, N S Key
No abstract text is available yet for this article.
November 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29178149/measuring-activities-and-participation-in-persons-with-haemophilia-a-systematic-review-of-commonly-used-instruments
#9
REVIEW
M A Timmer, S C Gouw, B M Feldman, A Zwagemaker, P de Kleijn, M F Pisters, R E G Schutgens, V Blanchette, A Srivastava, J A David, K Fischer, J van der Net
INTRODUCTION: Monitoring clinical outcome in persons with haemophilia (PWH) is essential in order to provide optimal treatment for individual patients and compare effectiveness of treatment strategies. Experience with measurement of activities and participation in haemophilia is limited and consensus on preferred tools is lacking. AIM: The aim of this study was to give a comprehensive overview of the measurement properties of a selection of commonly used tools developed to assess activities and participation in PWH...
November 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29168609/the-increasing-maturity-of-the-von-willebrand-factor-collagen-binding-in-von-willebrand-disease-diagnosis
#10
E J Favaloro, S Mohammed, S Oliver
No abstract text is available yet for this article.
November 23, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29168597/reliability-and-clinical-features-associated-with-the-ipsg-mri-tibiotalar-and-subtalar-joint-scores-in-children-adolescents-and-young-adults-with-haemophilia
#11
T Brunel, S Lobet, K Deschamps, C Hermans, K Peerlinck, J Vandesande, J-B Pialat
OBJECTIVES: To assess the reliability of the IPSG MRI scale for tibiotalar (TTJ) and subtalar joint (STJ) changes in young haemophilic patients, correlating MRI findings with functional scores and 3D-rearfoot kinematics. METHODS: A total of 37 haemophilic patients underwent bilateral MRI of the footankle, clinical evaluation and quantitative assessment of their 3D-rearfoot kinematics during walking. TTJ and STJ soft tissues were assessed twice along with osteochondral changes by two radiologists using the IPSG MRI scale...
November 23, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29168278/a-role-for-intravenous-immunoglobulin-in-the-treatment-of-acquired-von-willebrand-syndrome-associated-with-igm-gammopathy
#12
LETTER
M Lavin, K Ryan, B White, M Byrne, N M O'Connell, J S O'Donnell
No abstract text is available yet for this article.
November 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29168270/a-comparative-evaluation-of-a-new-fully-automated-assay-for-von-willebrand-factor-collagen-binding-activity-to-an-established-method
#13
F Stufano, L Baronciani, D Mane-Padros, G Cozzi, S Faraudo, F Peyvandi
INTRODUCTION: Laboratory diagnosis of von Willebrand disease (VWD) is made by the measurement of von Willebrand factor (VWF) protein level and its activities. Current VWF activity tests include ristocetin cofactor and collagen binding (VWF:CB) assays. AIM: We have undertaken an evaluation of a new fully automated VWF:CB assay relative to an established enzyme-linked immunosorbent assay (ELISA) method. METHODS: The two analytical systems operate with different detection principles: a chemiluminescent method performed on ACL AcuStar Analyzer (the former) and a colorimetric ELISA by Asserachrom Stago (the latter) (type III collagen from human placenta)...
November 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29148258/influence-of-medical-insurance-schemes-and-charity-assistance-projects-on-regular-prophylaxis-treatment-of-the-boys-with-severe-haemophilia-a-in-china
#14
Z Li, J Wu, Y Zhao, R Liu, K Li, Y Zhou, R Wu, R Yang, X Zhang, S Lian, Q Hu, X Li, J Gu, R Zhou, J Sun, C Li, W Xu, M-C Poon, J Xiao
OBJECTIVE: To explore the influence of medical insurance policy and charity assistance projects on the uptake and discontinuation of regular prophylaxis treatment in Chinese severe haemophilia A children. METHODOLOGY: This retrospective study was conducted on children with severe haemophilia A, who received FVIII prophylaxis treatment at 12 haemophilia centres in China from 1 November 2007 to 31 May 2013. RESULTS: The average duration of prophylaxis treatment received by haemophilia children significantly increased from 16...
November 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29148155/identifying-biomechanical-gait-parameters-in-adolescent-boys-with-haemophilia-using-principal-component-analysis
#15
L B Suckling, D Stephensen, M C Cramp, R Mahaffey, W I Drechsler
INTRODUCTION: Improvements in the medical management for those with haemophilia have resulted in improved clinical outcomes. However, current treatment regimens do not alleviate all joint haemarthroses with the potential for long-term joint deterioration remaining. The evaluation of functional activities such as gait, using standardized tools to monitor children with haemophilia is emerging. AIM: This study explored differences in sagittal plane biomechanics of walking in adolescent boys aged 11-18 years with haemophilia and an age-matched group of typically developing boys...
November 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29131461/somatosensory-profile-of-patients-with-haemophilia
#16
S Krüger, M K Boettger, T Hilberg
INTRODUCTION: Patients with haemophilia (PwH) suffer from an enhanced pain sensitivity due to repetitive joint bleedings. A comprehensive, quantitative examination of the somatosensory system has not been performed in this population to date. MATERIAL AND METHODS: Thirty patients with moderate or severe haemophilia A or B and 30 healthy controls were examined by means of Quantitative Sensory Testing to assess the function of the somatosensory system. Detection (DT) and pain thresholds (PT) were determined, amounting to a total of 13 parameters...
November 13, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29119711/bernard-soulier-syndrome-in-pakistan-biochemical-and-molecular-analyses-leading-to-identification-of-a-novel-mutation-in-gp1ba
#17
LETTER
D Böckelmann, A Naz, M Y J Siddiqi, E Lerner, K Sandrock-Lang, T S Shamsi, B Zieger
No abstract text is available yet for this article.
November 9, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29115006/type-2n-von-willebrand-disease-characterization-and-diagnostic-difficulties
#18
A Casonato, E Galletta, L Sarolo, V Daidone
INTRODUCTION: An abnormal factor VIII (FVIII) binding capacity of von Willebrand factor (VWF) identifies type 2N von Willebrand disease (VWD). Type 2N VWD patients are identified by means of the VWF FVIII binding (VWF:FVIIIB) assay, and especially their VWF:FVIIIB/VWF:Ag ratio (VWF:FVIIIB ratio). AIM: We report on our 15-year experience of diagnosing type 2N VWD. METHODS: We have performed 2178 VWF:FVIIIB assays in bleeders and normal subjects...
November 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29105952/congenital-factor-v-deficiency-and-decreased-vwf-in-a-chinese-male-patient-with-hematuria
#19
LETTER
X Wang, N Tang, Y Lu, D Li
No abstract text is available yet for this article.
November 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29082639/improved-joint-health-in-subjects-with-severe-haemophilia-a-treated-prophylactically-with-recombinant-factor-viii-fc-fusion-protein
#20
J Oldenburg, R Kulkarni, A Srivastava, J N Mahlangu, V S Blanchette, E Tsao, B Winding, J Dumont, N Jain
INTRODUCTION: Joint arthropathy is the long-term consequence of joint bleeding in people with severe haemophilia. AIM: This study assessed change in joint health over time in subjects receiving recombinant factor VIII Fc fusion protein (rFVIIIFc) prophylaxis. METHODS: ALONG is the phase 3 pivotal study in which the benefit of rFVIIIFc as a prophylactic treatment for bleeding control was shown in previously treated severe haemophilia patients ≥12 years of age (arm 1: 25-65 IU/kg every 3-5 days, arm 2: 65 IU/kg weekly and arm 3: episodic)...
October 30, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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