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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/28326647/exploring-some-intersections-between-pharmacokinetics-factor-viii-measurement-and-human-morphometrics-impact-of-recent-advances-in-haemophilia-study-design-on-our-understanding-of-optimal-haemophilia-treatment
#1
A Iorio, A N Edginton
No abstract text is available yet for this article.
March 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28321962/psychosocial-care-for-children-with-haemophilia-and-their-parents-in-the-netherlands
#2
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/28314057/acquired-von-willebrand-syndrome-in-inflammatory-bowel-disease
#3
LETTER
A Di Sabatino, C Ambaglio, N Aronico, N Ghidelli, M V Lenti, G Gamba, G R Corazza
No abstract text is available yet for this article.
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306218/implementation-of-a-recombinant-factor-ix-fc-fusion-protein-extended-infusion-desensitization-protocol
#4
LETTER
A M Clough, J A Gilreath, J P McPherson, N C Link, G M Rodgers, D Nance
No abstract text is available yet for this article.
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306216/molecular-diagnosis-of-haemophilia-a-in-patients-from-senegal
#5
LETTER
M Seck, C Costa, B F Faye, D Sy Bah, S A Touré, N Dieng, A Sall, M Gadji, A O Touré, D Lasne, C Rothschild, S Diop
No abstract text is available yet for this article.
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306198/plasma-levels-of-plasminogen-activator-inhibitor-1-and-bleeding-phenotype-in-patients-with-von-willebrand-disease
#6
S Abdul, J Boender, J J M C Malfliet, J Eikenboom, K Fijn van Draat, E P Mauser-Bunschoten, K Meijer, J de Meris, B A P Laros-van Gorkom, J G van der Bom, F W G Leebeek, D C Rijken, S Uitte de Willige
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. AIM: To investigate whether plasminogen activator inhibitor-1 (PAI-1) level influences the variation in bleeding tendency in VWD patients...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306191/biomechanical-markers-and-theoretical-concepts-related-to-haemophilic-ankle-and-subtalar-joint-arthropathy-introducing-the-term-haemophilic-tarsal-pan-arthropathy
#7
REVIEW
S Lobet, A McCarthy, C Hermans, K Peerlinck, G A Matricali, F Staes, K Deschamps
BACKGROUND: Although regular factor replacement can reduce the incidence of joint bleeds and slow down the development of haemophilic arthropathy, the ankle joint remains particularly vulnerable even in children with haemophilia on primary or secondary prophylaxis and is now the primary joint affected. The heterogeneity in the pathoaetiology of haemophilic ankle arthropathy means that the functional consequences of early stage of ankle arthropathy are difficult to define as early morphological and structural changes can be observed in clinically asymptomatic ankles...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306188/protein-modelling-to-understand-fgb-mutations-leading-to-congenital-hypofibrinogenaemia
#8
A Casini, R Vilar, Y Beauverd, D Aslan, K Devreese, V Mondelaers, L Alberio, C Gubert, P de Moerloose, M Neerman-Arbez
INTRODUCTION: Congenital hypofibrinogenaemia is a quantitative fibrinogen disorder characterized by proportionally decreased levels of functional and antigenic fibrinogen. Mutations accounting for quantitative fibrinogen disorders are relatively frequent in the conserved COOH-terminal globular domains of the γ and Bβ chains. The latter mutations are of particular interest since the Bβ-chain is considered the rate-limiting chain in the hepatic production of the fibrinogen hexamer. AIM: The aim of this study was to study the molecular pattern of four patients with congenital hypofibrinogenaemia...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306186/sippet-methodology-analysis-and-generalizability
#9
REVIEW
F Peyvandi, P M Mannucci, R Palla, F R Rosendaal
The development of anti-FVIII neutralizing alloantibodies (inhibitors), occurring in about one-third of previously untreated patients (PUPs) with severe haemophilia A, depends on various genetic and environmental risk factors. Several previous studies have reported on the immunogenicity of FVIII concentrates, and due to differences in study design, study period, inhibitor testing frequency and follow-up duration the results were inconclusive. The first randomized trial on this unresolved question (SIPPET) included 251 previously untreated or minimally treated patients with severe haemophilia A treated with either a single plasma-derived FVIII (pdFVIII) containing VWF or a recombinant FVIII (rFVIII)...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306181/total-ankle-replacement-in-patients-with-haemophilic-arthropathy-primary-arthroplasty-and-conversion-of-painful-ankle-arthrodesis-to-arthroplasty
#10
M Preis, T Bailey, M Jacxsens, A Barg
INTRODUCTION: There are two general surgical approaches for operative treatment of end-stage haemophilic ankle arthropathy: ankle arthrodesis and total ankle replacement (TAR). AIM: The aim of this study was to determine intraoperative and postoperative complications and evaluate the mid-term clinical and radiographic outcomes of TAR in patients with haemophilic arthropathy. METHODS: Fourteen patients with a mean age of 51.4 ± 10.2 years (range = 32...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28233431/emergency-department-utilization-by-haemophilia-patients-in-united-states
#11
A Zakieh, A H Siddiqui
INTRODUCTION: Patients with haemophilia are prone to medical emergencies. Emergency departments (ED) often do not have a haematologist on-call for consultation. AIM: The aim of this study was to determine the utilization of ED by patients with haemophilia. METHODS: Retrospective review of the 'National ED Database Sample' for years 2006-2013 was performed. Patient and hospital demographics were collected. Hospital charges were projected to national levels using discharge-weighted variables...
February 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28233383/indirect-comparisons-of-efficacy-and-weekly-factor-consumption-during-continuous-prophylaxis-with-recombinant-factor-viii-fc-fusion-protein-and-conventional-recombinant-factor-viii-products
#12
A Iorio, S Krishnan, K J Myrén, S Lethagen, N McCormick, S Yermakov, P Karner
INTRODUCTION: Recombinant factor VIII (rFVIII) products with extended half-lives have the potential to improve adherence and outcomes in haemophilia beyond the results obtained with conventional rFVIII products. AIM: In the absence of head-to-head comparisons, annualized bleed rates (ABRs) and weekly factor consumption with rFVIII Fc fusion protein (rFVIIIFc) and conventional rFVIII products were indirectly compared using studies of continuous prophylaxis. METHODS: A systematic literature review was conducted to identify studies of rFVIII products for comparison with rFVIIIFc in the continuous prophylactic treatment of previously treated adolescents and adults with moderate and severe haemophilia A...
February 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28233381/pharmacokinetics-of-a-novel-extended-half-life-glycopegylated-factor-ix-nonacog-beta-pegol-n9-gp-in-previously-treated-patients-with-haemophilia-b-results-from-two-phase-3-clinical-trials
#13
A Tiede, F Abdul-Karim, M Carcao, P Persson, W H O Clausen, S Kearney, T Matsushita, C Negrier, J Oldenburg, E Santagostino, G Young
INTRODUCTION: Nonacog beta pegol (N9-GP) is a glycoPEGylated recombinant factor IX (FIX) with an extended half-life developed for routine prophylaxis and the prevention and treatment of bleeding episodes in patients with haemophilia B. AIM: The aim of this study was to evaluate the pharmacokinetics (PK) of N9-GP. METHODS: Data from 41 previously treated haemophilia B patients, enrolled globally (16 adolescents/adults and 25 children; FIX activity ≤0...
February 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220685/incidence-of-low-titre-factor-viii-inhibitors-in-patients-with-haemophilia-a-meta-analysis-of-observational-studies
#14
A Messori, F Peyvandi, D Mengato, P M Mannucci
INTRODUCTION: A few studies have been focused on low-titre inhibitors in patients with haemophilia A. Although several putative factors have been implicated in the development of these inhibitors, solid data are still lacking. AIM: The aim of this study was to perform a proportion meta-analysis on the incidence of low-titre inhibitors in haemophilia A. METHODS: We surveyed the PubMed database to identify studies on de novo development of low-titre inhibitors in haemophilia A patients...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220631/treatment-of-bleeding-episodes-with-recombinant-factor-viii-fc-fusion-protein-in-a-long-study-subjects-with-severe-haemophilia-a
#15
A D Shapiro, J N Mahlangu, D Perry, J Pasi, D V Quon, P Chowdary, E Tsao, S Li, A Innes, G F Pierce, G A Allen
INTRODUCTION: The Phase 3 A-LONG study demonstrated the safety and efficacy of rFVIIIFc for the control and prevention of bleeding episodes in severe haemophilia A. AIM: To describe the treatment of bleeding episodes with rFVIIIFc in the A-LONG study. METHODS: A-LONG subjects (<1 IU dL(-1) endogenous FVIII) were treated with individualized prophylaxis (Arm 1), weekly prophylaxis (Arm 2) or episodic treatment (Arm 3). Information recorded for each bleeding episode included type, location and dose to treat the episode...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220580/molecular-diagnosis-of-von-willebrand-disease
#16
REVIEW
L Baronciani, A Goodeve, F Peyvandi
The role of molecular characterization in the diagnosis of von Willebrand disease (VWD) is not essential if the patients have been extensively investigated using phenotypic analysis. On the other hand, if some of these phenotype assays are not available, the identification of the mutation causing the disease could be crucial for an accurate diagnosis. Nevertheless, there are several reasons for performing molecular analysis in patients phenotypically well characterized, e.g. to identify the mutation causing VWD can be useful for patients and their family members when prenatal diagnosis is required (type 3 or severe type 2)...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220573/a-comparison-of-two-types-of-ankle-supports-in-men-with-haemophilia-and-unilateral-ankle-pain-from-arthropathy
#17
D Oleson, L Fox, T Nguyen, P Sochacki, M McCarthy, E Adams, M Recht
INTRODUCTION: Adults with haemophilia frequently have a painful and disordered gait due to ankle arthropathy. AIM: The aim of this study was to determine if pain and gait parameters were affected by the use of different types of ankle bracing. METHODS: We investigated the gait patterns of 17 men with severe haemophilia without bracing, using a fracture boot (FB) and a carbon fibre floor reaction ankle foot orthosis (CF-AFO). Pain relief was determined in each condition...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220572/a-prevalent-ctla4-missense-variant-significantly-associates-with-inhibitor-development-in-argentine-patients-with-severe-haemophilia-a
#18
LETTER
V D Marchione, J R Zuccoli, M M Abelleyro, C P Radic, D Neme, M Candela, M de Tezanos Pinto, C D De Brasi, L C Rossetti
No abstract text is available yet for this article.
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220559/research-and-policy-implications-of-a-recently-published-controlled-study-in-previously-untreated-haemophilia-patients-at-high-risk-of-inhibitor-development
#19
LETTER
A Iorio
No abstract text is available yet for this article.
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220555/population-pharmacokinetic-characterization-of-bay-81-8973-a-full-length-recombinant-factor-viii-lessons-learned-importance-of-including-samples-with-factor-viii-levels-below-the-quantitation-limit
#20
D Garmann, S McLeay, A Shah, P Vis, M Maas Enriquez, B A Ploeger
INTRODUCTION: The pharmacokinetics (PK), safety and efficacy of BAY 81-8973, a full-length, unmodified, recombinant human factor VIII (FVIII), were evaluated in the LEOPOLD trials. AIM: The aim of this study was to develop a population PK model based on pooled data from the LEOPOLD trials and to investigate the importance of including samples with FVIII levels below the limit of quantitation (BLQ) to estimate half-life. METHODS: The analysis included 1535 PK observations (measured by the chromogenic assay) from 183 male patients with haemophilia A aged 1-61 years from the 3 LEOPOLD trials...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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