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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/28092925/risk-based-management-of-dental-procedures-in-patients-with-inherited-bleeding-disorders-development-of-a-dental-bleeding-risk-assessment-and-treatment-tool-debratt
#1
L Rasaratnam, P Chowdary, D Pollard, B Subel, C Harrington, U R Darbar
INTRODUCTION: Successful outcomes in dental management for patients with inherited bleeding disorders require close collaboration between haematology teams and dentists. AIM: To review outcomes of an interdisciplinary pathway for dental procedures by assessing adequacy and appropriateness of haemostatic management. METHODS: Two hundred dental procedures in 30 patients with inherited bleeding disorders were included. A Dental Bleeding Risk Assessment and Treatment Tool (DeBRATT) was developed to identify four categories of bleeding risk (no risk, low, moderate and high risk of bleeding) in relation to the severity of the bleeding disorder and the invasiveness of dental procedure...
January 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28092924/evaluation-of-the-use-of-rotational-thromboelastometry-in-the-assessment-of-fxi-deficency
#2
G N Pike, A M Cumming, J Thachil, C R M Hay, P H B Bolton-Maggs, J Burthem
INTRODUCTION: The absence of a reliable clinical test to predict bleeding tendency leaves factor XI (FXI)-deficient individuals at risk of overtreatment or under treatment. AIM: To assess whether rotational thromboelastometry has value in detection of FXI deficiency and identification of bleeding tendency. METHODS: Thromboelastometry was measured in whole blood and platelet-rich plasma (PRP) samples containing corn trypsin inhibitor (CTI) from controls (n = 50) and FXI-deficient individuals (n = 93) at tissue factor (TF) 0...
January 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28083987/vwf-collagen-types-iii-and-vi-binding-defects-in-a-cohort-of-type-2m-vwd-patients-a-strategy-for-improvement-of-a-challenging-diagnosis
#3
LETTER
T Fidalgo, A Oliveira, C Silva Pinto, P Martinho, G Ferreira, R Salvado, T Sevivas, C Catarino, M L Ribeiro
No abstract text is available yet for this article.
January 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28026130/efficacy-and-safety-of-a-vwf-fviii-concentrate-wilate-%C3%A2-in-inherited-von-willebrand-disease-patients-undergoing-surgical-procedures
#4
A Srivastava, M Serban, S Werner, B A Schwartz, C M Kessler
INTRODUCTION: Surgical procedures in von Willebrand disease (VWD) patients may require prophylactic treatment with exogenous von Willebrand factor (VWF) and coagulation factor VIII (FVIII) to prevent excessive bleeding. Wilate(®) is a plasma-derived, double virus-inactivated, highly purified, freeze-dried VWF/FVIII concentrate, containing both factors in a physiological activity ratio of 1:1. AIM: To investigate the efficacy and safety of wilate(®) in maintaining haemostasis in VWD patients undergoing surgical procedures...
December 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28026074/measurement-properties-of-the-haem-a-qol-in-haemophilia-clinical-trials
#5
S von Mackensen, A Eldar-Lissai, P Auguste, S Krishnan, R von Maltzahn, R Yu, K W Wyrwich
INTRODUCTION: Patients with haemophilia on long-acting prophylactic treatment may experience an improvement in health-related quality of life (HRQoL) through reductions in breakthrough bleeds and associated complications, including long-term joint damage, compared with episodic treatment. AIM: This analysis examined clinical trial data to understand the psychometric characteristics (reliability, validity and sensitivity to change over time) of the Haem-A-QoL Questionnaire in adult males with haemophilia...
December 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28026073/use-of-the-ukhcdo-database-for-a-postmarketing-surveillance-study-of-different-doses-of-recombinant-factor-viia-in-haemophilia
#6
C R M Hay, T Sharpe, G Dolan
INTRODUCTION: Recombinant factor VIIa (rFVIIa) is recommended in Europe at standard (3 × 90 μg kg(-1) ) or high (1 × 270 μg kg(-1) ) doses. When granting the license for the high dose, the European Medicines Agency (EMA) requested postmarketing surveillance for thrombosis. This was conducted by the United Kingdom National Haemophilia Database (NHD) on behalf of Novo Nordisk and the EMA. AIM: To assess the use and safety of rFVIIa utilizing prospective data collected by the NHD (1 January 2008 to 30 June 2011)...
December 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27995737/splice-site-mutation-in-factor-x-gene-manifesting-as-severe-intracranial-haemorrhage-in-neonatal-period-with-a-challenging-treatment-course
#7
LETTER
P P Madhusoodhan, B Y Lu, J Chen, C L Jones, J A Meyer, E A Roman, M Nardi, W L Carroll, T Bhatla
No abstract text is available yet for this article.
December 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27995727/kinetic-analysis-and-binding-studies-of-a-new-recombinant-human-factor-viia-for-treatment-of-haemophilia
#8
J Grandoni, G Perret, C Forier
INTRODUCTION/AIM: LR769 is a new second-generation recombinant human Factor VIIa (rhFVIIa) developed for haemophilia treatment. We determined enzymatic properties of LR769 and its interaction with antithrombin, tissue factor, platelets and endothelial protein C receptor (EPCR), compared with NovoSevenRT. METHODS: Kinetic enzyme assays and active site titration were used for enzymatic studies. Surface Plasmon Resonance (SPR) was used for determination of binding constants...
December 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27995680/bay-81-8973-a-full-length-recombinant-factor-viii-manufacturing-processes-and-product-characteristics
#9
REVIEW
S Garger, J Severs, L Regan, A Hesslein, J Ignowski, P Wu, E Long, S Gupta, S Liu, W Wang
BAY 81-8973 (Kovaltry(®) , Bayer, Berkeley, CA, USA) is an unmodified, full-length recombinant human factor VIII (FVIII) approved for prophylaxis and on-demand treatment of bleeding episodes in patients with haemophilia A. The BAY 81-8973 manufacturing process is based on the process used for sucrose-formulated recombinant FVIII (rFVIII-FS), with changes and enhancements made to improve production efficiency, further augment pathogen safety, and eliminate animal- and human-derived raw materials from the production processes...
December 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27995677/plasma-products-do-not-solve-the-inhibitor-problem
#10
LETTER
H M van den Berg, S Pipe, R Ljung
No abstract text is available yet for this article.
December 19, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27976460/interventional-therapies-and-in-hospital-outcomes-in-acute-coronary-syndromes-complicated-by-von-willebrand-disease
#11
P F Fogarty, A Blair, R Vega, W H Matthai, P A Gimotty
INTRODUCTION: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. AIM: Investigate the impact of the VWD bleeding tendency on in-hospital management of acute coronary syndromes (ACS). METHODS: Using discharge data from the National Inpatient Sample (NIS), the features of presentation and in-hospital treatment among ACS hospital discharges with and without a VWD diagnosis were investigated. A total of 264 case discharges and 705 860 control discharges were identified...
December 14, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27943561/transmission-of-severe-haemophilia-a-by-in-vitro-fertilization-with-donated-eggs
#12
LETTER
C Sweeney, L Khalil, N Curry
No abstract text is available yet for this article.
December 11, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27943552/usefulness-of-a-simple-self-administered-joint-condition-assessment-sheet-to-predict-the-need-for-orthopaedic-intervention-in-the-management-of-haemophilic-arthropathy
#13
T Masaoka, K Amano, H Takedani, T Suzuki, M Otaki, I Seita, T Tateiwa, T Shishido, K Yamamoto, K Fukutake
INTRODUCTION: Detecting signs of joint deterioration is important for early effective orthopaedic intervention in managing haemophilic arthropathy. AIM: We developed a simple, patient self-administered sheet to evaluate the joint condition, and assessed the predictive ability of this assessment sheet for the need for an orthopaedic intervention. METHODS: This was a single-centre, cross-sectional study. The association between the score of each of the four items of the assessment sheet (bleeding, swelling, pain and physical impairment) and the results of radiological findings and physical examinations based on Haemophilia Joint Health Score 2...
December 11, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27943467/low-bleeding-rates-with-increase-or-maintenance-of-physical-activity-in-patients-treated-with-recombinant-factor-viii-fc-fusion-protein-rfviiifc-in-the-a-long-and-kids-a-long-studies
#14
LETTER
D V Quon, R Klamroth, R Kulkarni, A D Shapiro, R I Baker, G Castaman, B A Kerlin, E Tsao, G Allen
No abstract text is available yet for this article.
December 11, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928900/determining-the-health-related-quality-of-life-in-individuals-with-haemophilia-in-developing-economies-results-from-the-brazilian-population
#15
T Salomon, D G Chaves, S Brener, P R J Martins, J V M Mambrini, S V Peixoto
INTRODUCTION: Several studies show the negative impact of haemophilia in health-related quality of life (HRQOL). This issue is not well explored in developing countries. OBJECTIVES: This cross-sectional study aimed to evaluate the HRQOL and its associated factors in patients with haemophilia A/B in Brazil. Data were collected by questionnaire and in medical records, including a Portuguese version of Haem-A-Qol. RESULTS: Brazilian patients were invited to the study and 175 participants (147 haemophilics A and 28 haemophilics B) were included...
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928886/f376a-m388a-solulin-a-new-promising-antifibrinolytic-for-severe-haemophilia-a
#16
J Parcq, K U Petersen, A Borel-Derlon, P Gautier, M Ebel, D Vivien, Y Repessé
INTRODUCTION: Haemophilia is a major bleeding disorder due to a deficiency of procoagulant factor VIII (type A) or IX (type B). The treatment is substitutive and based on infusion of factor concentrates. Main limitations of this therapy are cost, short factor half-life and the development of inhibitors (up to 30% of severe HA patients). An important aggravating factor of haemophilia is due to a premature fibrinolysis, directing attention to the therapeutic potential of suitable antifibrinolytics...
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928870/balance-evaluation-in-haemophilic-preadolescent-patients-using-nintendo-wii-balance-board-%C3%A2
#17
S Pérez-Alenda, J J Carrasco, M Aguilar-Rodríguez, L Martínez-Gómez, M Querol-Giner, R Cuesta-Barriuso, A Torres-Ortuño, F Querol
INTRODUCTION: Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. AIM: This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board(®) (WBB). METHODS: Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study...
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928869/possible-assessment-of-coagulation-function-and-haemostasis-therapy-using-comprehensive-coagulation-assays-in-a-patient-with-acquired-haemophilia-a
#18
LETTER
M Takeyama, K Nogami, T Matsumoto, M Taguchi, K Yada, N Okahashi, I Amano, H Kimura, M Shima
No abstract text is available yet for this article.
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928863/do-the-sippet-study-results-apply-to-the-patients-i-treat
#19
LETTER
K Fischer, J Blatny
No abstract text is available yet for this article.
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27928848/prevalence-and-predictors-of-loss-to-follow-up-in-young-adults-with-mild-haemophilia
#20
LETTER
H L Sun, K McIntosh, C Woo, E Crilly, C Bartholomew, D Gue, L De Marchi, S Squire, A Silva, M Yang, J K Wu, S Jackson
No abstract text is available yet for this article.
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
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