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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/27891721/extended-half-life-pegylated-full-length-recombinant-factor-viii-for-prophylaxis-in-children-with-severe-haemophilia-a
#1
E S Mullins, O Stasyshyn, M T Alvarez-Román, D Osman, R Liesner, W Engl, M Sharkhawy, B E Abbuehl
INTRODUCTION: Primary factor VIII (FVIII) prophylaxis is the optimal treatment in children with severe haemophilia A. They are expected to benefit from extended half-life (T1/2 ) FVIII coverage by reduced infusion frequency while maintaining haemostatic efficacy. AIMS: To determine immunogenicity, pharmacokinetics (PK), efficacy, safety and quality of life of prophylaxis with a polyethylene glycol (peg)-ylated FVIII (BAX 855) based on full-length recombinant FVIII (ADVATE) in paediatric previously treated patients (PTPs) with severe haemophilia A...
November 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27891716/clinical-characteristics-of-two-patients-with-%C3%AE-1-antitrypsin-pittsburgh-in-a-chinese-family
#2
LETTER
L Cao, Z Wang, X Bai, Z Yu, J Su, C Ruan
No abstract text is available yet for this article.
November 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27860135/joint-lavage-followed-by-viscosupplementation-and-triamcinolone-in-patients-with-severe-haemophilic-arthropathy-objective-functional-results
#3
M U Rezende, F R Andrusaitis, R T Silva, E Okazaki, J D A Carneiro, G C Campos, A F Pailo, R Frucchi, T Pasqualin, P R Villaça
INTRODUCTION: Viscosupplementation can improve function in haemophilia patients. Viscosupplementation results can be improved by prior joint lavage and triamcinolone administration. AIM: To objectively assess whether viscosupplementation and associated triamcinolone use in patients with severe haemophilic arthropathy following joint lavage improves force and balance and reduces bleeding events. METHODS: Fourteen patients with haemophilic knee arthritis with and without the involvement of other joints underwent joint lavage and subsequent injections of Hylan G-F20 and triamcinolone into all affected joints...
November 16, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27862691/evaluation-of-the-use-of-global-haemostasis-assays-to-monitor-treatment-in-factor-xi-deficiency
#4
G N Pike, A M Cumming, J Thachil, C R M Hay, J Burthem, P H B Bolton-Maggs
INTRODUCTION: Previous guidelines recommend that FXI:C levels should be used to monitor FXI replacement in factor XI (FXI) deficiency. However, FXI:C levels do not correlate with bleeding tendency in this disorder and may not be the optimal test by which to monitor and determine further treatment in the postoperative period. AIM: To assess whether the thrombin generation assay (TGA) and rotational thromboelastometry can be used to monitor FXI replacement peri-operatively in FXI deficiency and to determine if changes in FXI:C levels correlate with changes in thrombin generation and clot formation parameters following treatment with solvent-detergent fresh frozen plasma (SD-FFP)...
November 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27862687/anti-factor-viii-antibodies-in-brothers-with-haemophilia-a-share-similar-characteristics
#5
J Kahle, A Orlowski, D Stichel, J F Healey, E T Parker, S M Donfield, J Astermark, E Berntorp, P Lollar, D Schwabe, C Königs
INTRODUCTION: The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is currently the most serious complication for patients with haemophilia A undergoing FVIII replacement therapy. Several genetic factors have been acknowledged as risk factors for inhibitor development. AIM: To analyze the influence of genetic factors on the nature of the humoral immune response to FVIII in eight brother pairs with inhibitors. METHODS: The domain specificity of FVIII-specific IgG was analysed by antibody binding to FVIII fragments and homologue-scanning mutagenesis (HSM)...
November 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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
#6
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/27813318/bleeding-and-liver-transplantation-outcomes-in-haemophilia
#7
K D Mehta, M V Ragni
BACKGROUND: Hepatitis C is the major cause of end-stage liver disease and the major indication for orthotopic liver transplantation (OLTx) in individuals with haemophilia. AIM: To assess the epidemiology and outcomes of OLTx in U.S. haemophilia patients. METHODS: We investigated haemophilia liver transplant recipients between 1993 and 2012, using the Nationwide Inpatient Sample, identified by ICD9 code 50.59. RESULTS: Of the 11 267 (weighted n = 54 691) patients undergoing OLTx, 44 (0...
November 4, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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
#8
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...
November 4, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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
#9
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/27785858/a-prospective-diagnostic-accuracy-study-evaluating-rotational-thromboelastometry-and-thromboelastography-in-100-patients-with-von-willebrand-disease
#10
D E Schmidt, A Majeed, M Bruzelius, J Odeberg, M Holmström, A Ågren
INTRODUCTION: Rotational thromboelastometry (ROTEM(®) ) and thromboelastography (TEG(®) ) are increasingly used in the perioperative and emergency assessment of bleeding tendencies. The diagnostic value of ROTEM and TEG for von Willebrand disease (VWD) remains to be established. AIM: To investigate whether ROTEM and TEG can discriminate patients with VWD from healthy controls. METHODS: Rotational thromboelastometry and TEG whole blood coagulation profiles were compared between VWD patients (n = 100) and healthy controls (n = 89)...
October 26, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27762081/pilot-study-on-18-f-fdg-pet-ct-for-detection-of-inflammatory-changes-in-blood-induced-knee-arthropathy-in-a-rabbit-model
#11
A Shammas, A S Doria, A Amirabadi, H Gahunia, R Jong, M Charron, R Moineddin, U Metser
RATIONALE: (18) F-FDG-PET/CT has a potential role in the early detection of haemophilic arthritis, at a time when treatment may still avoid further joint degeneration. The purposes of this pilot study were to determine the ability of (18) F-FDG-PET/CT to detect inflammatory changes associated with blood-induced arthropathy in knees of a rabbit model. METHODS: Ten juvenile rabbits were imaged at baseline and weeks 5 and 17 post intraarticular autologous blood injections (ABI)...
October 20, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27761964/recombinant-b-domain-deleted-porcine-sequence-factor-viii-r-pfviii-for-the-treatment-of-bleeding-in-patients-with-congenital-haemophilia-a-and-inhibitors
#12
J N Mahlangu, T A Andreeva, D E Macfarlane, C Walsh, N S Key
INTRODUCTION: Development of inhibitors to human FVIII (hFVIII) significantly complicates the control of bleeding events in patients with haemophilia A. AIM: This prospective, multicentre, open-label, non-comparative, Phase II study evaluated the haemostatic activity of a recombinant B-domain-deleted porcine FVIII (r-pFVIII), in the treatment of non-life/non-limb-threatening bleeding in individuals with haemophilia A and FVIII inhibitors. METHODS: Acute bleeding episodes in patients with pFVIII inhibitor titres <0...
October 20, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27761962/european-retrospective-study-of-real-life-haemophilia-treatment
#13
E Berntorp, G Dolan, C Hay, S Linari, E Santagostino, A Tosetto, G Castaman, M T Álvarez-Román, R Parra Lopez, J Oldenburg, T Albert, U Scholz, M Holmström, J-F Schved, M Trossaërt, C Hermans, A Boban, C Ludlam, S Lethagen
INTRODUCTION: Haemophilia treatment varies significantly between individuals, countries and regions and details of bleed rates, factor consumption and injection frequency are often not available. AIM: To provide an overview of the FVIII/FIX treatment practice and outcome for patients with haemophilia A (HA) or haemophilia B (HB) across Europe. METHODS: Non-interventional, 12-month retrospective study where anonymized data were retrieved from haemophilia centres/registers in Belgium, France, Germany, Italy, Spain, Sweden and the United Kingdom...
October 20, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27726259/rct-subjective-physical-performance-and-quality-of-life-after-a-6-month-programmed-sports-therapy-pst-in-patients-with-haemophilia
#14
B Runkel, S Von Mackensen, T Hilberg
INTRODUCTION: Musculoskeletal bleedings lead to limitations in the locomotor system and consequently, in health-related quality of life (HRQoL) in patients with haemophilia (PwH). Sports therapy is increasingly recommended to improve their physical performance. Until today, randomised controlled studies investigating changes in physical performance in PwH are rare. AIM: This study investigates the impact of programmed sports therapy on the subjective physical performance and the HRQoL in PwH...
October 11, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27704637/retrospective-analysis-of-in-vivo-recovery-and-clearance-during-continuous-infusion-of-recombinant-factor-viii-products-a-single-institution-study
#15
N Suzuki, A Hirakawa, M Kishimoto, T Kanematsu, M Ogawa, H Kiyoi, T Matsushita
BACKGROUND: Continuous infusion (CI) of recombinant FVIII (rFVIII) concentrates has been reported as an effective and safe method to achieve haemostasis during major surgeries or severe bleeding events. For more effective and safer CI, better understanding of in vivo recovery (IVR) and clearance (CL) issues is imperative. OBJECTIVE: We investigated the following factors affecting IVR and CL using univariate and multivariate regression analyses during 47 CIs in 34 patients: rFVIII concentrate type, haemophilia severity, blood type, the presence of hepatitis C virus (HCV) or human immunodeficiency virus (HIV), age and body mass index (BMI)...
October 5, 2016: 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
#16
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...
September 30, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27641050/the-impact-of-a-haemophilia-education-intervention-on-the-knowledge-and-health-related-quality-of-life-of-parents-of-indian-children-with-haemophilia
#17
S Phadnis, A Kar
INTRODUCTION: The impact of haemophilia education on the quality of life of parents of children with haemophilia from low income settings has not been studied. AIM: The purpose of this study was to determine the impact of an education intervention on health related quality of life (HRQOL) of parents of children with haemophilia, parent's knowledge about haemophilia and its management, and to determine whether education about haemophilia can positively impact these outcomes...
September 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27633342/choosing-outcome-assessment-tools-in-haemophilia-care-and-research-a-multidisciplinary-perspective
#18
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/27611596/visualization-of-haemophilic-arthropathy-in-f8-rats-by-ultrasonography-and-micro-computed-tomography
#19
K R Christensen, K Roepstorff, M Petersen, B Wiinberg, A K Hansen, M Kjelgaard-Hansen, L N Nielsen
INTRODUCTION: A major complication of haemophilia is haemophilic arthropathy (HA), a debilitating disorder with an incompletely defined pathobiology. High-resolution imaging may provide new knowledge about onset and progression of HA, and thereby support identification of new treatment opportunities. Recently, a F8(-/-) rat model of HA was developed. The size of the rat allows for convenient and high resolution imaging of the joints, which could enable in vivo studies of HA development...
September 9, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27611464/relative-contributions-of-bleeding-scores-and-iron-status-on-health-related-quality-of-life-in-von-willebrand-disease-a-cross-sectional-study
#20
Y Xu, M Deforest, J Grabell, W Hopman, P James
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Currently, studies investigating the health-related quality of life (HR-QoL) in VWD using standardized tools are limited, particularly among patients with mild decreases in von Willebrand factor or activity. AIM: To determine HR-QoL and its predictors among patients with mild, moderate and severe forms of VWD. METHODS: Patients with clinical diagnosis of VWD were recruited from a tertiary Inherited Bleeding Disorder Clinic...
September 9, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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