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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/28815880/immunogenicity-efficacy-and-safety-of-nuwiq-%C3%A2-human-cl-rhfviii-in-previously-untreated-patients-with-severe-haemophilia-a-interim-results-from-the-nuprotect-study
#1
R J Liesner, M Abashidze, O Aleinikova, C Altisent, M J Belletrutti, A Borel-Derlon, M Carcao, H Chambost, A K C Chan, L Dubey, J Ducore, N A Fouzia, M Gattens, Y Gruel, B Guillet, N Kavardakova, M El Khorassani, A Klukowska, T Lambert, S Lohade, M Sigaud, V Turea, J K M Wu, V Vdovin, A Pavlova, M Jansen, L Belyanskaya, O Walter, S Knaub, E J Neufeld
INTRODUCTION: Nuwiq(®) (Human-cl rhFVIII) is a fourth generation recombinant FVIII, produced in a human cell line, without chemical modification or protein fusion. No inhibitors developed in studies with Nuwiq(®) in 201 previously treated patients with haemophilia A (HA). The immunogenicity, efficacy and safety of Nuwiq(®) in previously untreated patients (PUPs) with severe HA are being assessed in the ongoing NuProtect study. METHODS: The study, conducted across 38 centres worldwide, is evaluating 110 true PUPs of all ages and ethnicities enrolled for study up to 100 exposure days (EDs) or 5 years maximum...
August 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28806864/study-of-physical-function-in-adolescents-with-haemophilia-the-so-fit-study
#2
K Khair, M Holland, M Bladen, A Griffioen, P McLaughlin, S von Mackensen
INTRODUCTION: Contemporary haemophilia care demands Patient-Reported Outcomes. SO-FIT is a UK multi-centre study, assessing self-reported function, health-related quality of life (HRQoL) and joint health in boys with severe haemophilia. METHODS: Subjective physical function (PedHAL, HEP-Test-Q) and HRQoL (Haemo-QoL Short Form [SF]) were assessed alongside joint health using the objective Haemophilia Joint Health Score (HJHS v2.1). Demographic and clinical data were collected...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28806860/patient-caregiver-and-provider-perceptions-of-pain-and-pain-management-in-adolescents-and-young-adults-with-bleeding-disorders
#3
Angela Lambing, Cynthia D Nichols, James E Munn, Terry L Anderson, Bartholomew J Tortella, Michelle L Witkop
INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28806858/the-haemtrack-home-therapy-reporting-system-design-implementation-strengths-and-weaknesses-a-report-from-uk-haemophilia-centre-doctors-organisation
#4
C R M Hay, H Xiang, M Scott, P W Collins, R Liesner, G Dolan, R Hollingsworth
INTRODUCTION: Haemtrack is an electronic home treatment diary for patients with inherited bleeding disorders, introduced in 2008. It aimed to improve the timeliness and completeness of patient-reported treatment records, to facilitate analysis of treatment and outcome trends. The system is easy to use, responsive and accessible. METHODS: The software uses Microsoft technologies with a SQL Server database and an ASP.net website front-end, running on personal computers, android and I-phones...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28782247/arthroscopic-debridement-for-advanced-haemophilic-ankle-arthropathy
#5
LETTER
T Yasui, J Hirose, K Ono, H Takedani
No abstract text is available yet for this article.
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28780772/prophylaxis-use-among-males-with-haemophilia-b-in-the-united-states
#6
M Ullman, Q C Zhang, S D Grosse, M Recht, J M Soucie
INTRODUCTION: Prophylaxis is considered the optimal treatment for persons with moderate to severe haemophilia (factor activity between 1-5% of normal and <1% of normal respectively) in countries where safe factor concentrates are available and economically feasible. Historically, prophylactic treatment has not been well studied in the haemophilia B (HB) population due to difficulties in obtaining a sufficiently large sample. AIM: This study examines the prevalence of prophylaxis use among a robust sample of persons with HB in the United States and its association with specific demographic and clinical characteristics...
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28780770/subcutaneous-diphtheria-and-tetanus-vaccines-in-children-with-haemophilia-a-pilot-study-and-review-of-the-literature
#7
B A Schaefer, R A Gruppo, E S Mullins, C Tarango
INTRODUCTION: Subcutaneous (SQ) vaccination has emerged as standard of care in children with severe bleeding disorders to reduce unnecessary factor exposure and avoid provoking an intramuscular bleed, but little is known about comparative immunogenicity to intramuscular (IM) vaccination. AIM: To confirm immunogenicity of Diphtheria Tetanus acellular Pertussis (DTaP) vaccines administered SQ to individuals <6 years old with haemophilia. METHODS: We performed a retrospective and prospective pilot study of tetanus and diphtheria antibody titres among patients evaluated at our Haemophilia Treatment Centre between 2015-2016...
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28780769/inherited-bleeding-disorders-and-zika-virus-infection
#8
LETTER
B Joob, V Wiwanitkit
No abstract text is available yet for this article.
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28780768/haemophilia-and-inflammatory-bowel-disease-we-still-know-very-little
#9
LETTER
Ł Dembiński, A Klukowska, A Banaszkiewicz
No abstract text is available yet for this article.
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28780767/ageing-successfully-with-haemophilia-a-multidisciplinary-programme
#10
E Boccalandro, M E Mancuso, S Riva, D M Pisaniello, F Ronchetti, E Santagostino, F Peyvandi, L P Solimeno, P M Mannucci, G Pasta
INTRODUCTION: Persons with haemophilia (PWH) born before the middle 1970s have spent a substantial part of their lives without the benefits of replacement therapy, that became available on a relative large scale only during the 1970s. As a consequence, this group of PWH, although still relatively young, suffers from musculoskeletal and functional problems that are typical of old people. METHODS: We report herewith the short-term results of a project based upon a multidisciplinary training programme led by a physiotherapist and an occupational therapist, that was implemented over a period of 12 months in 40 patients with severe or moderate hemophilia A or B born before the middle 1970s and regularly followed-up at a comprehensive haemophilia treatment centre in Italy...
August 6, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28776894/persept-1-a-phase-3-trial-of-activated-eptacog-beta-for-on-demand-treatment-of-haemophilia-inhibitor-related-bleeding
#11
M Wang, J B Lawrence, D V Quon, J Ducore, M L Simpson, L N Boggio, I S Mitchell, G Yuan, W A Alexander, J-F Schved
INTRODUCTION: Haemophilia A or B patients with inhibitors have been treated with FVIIa-containing bypassing agents for over 20 years. However, due to uncertainty regarding dose response and thrombotic risk, the use of a gradual, titrated, minimal dosing strategy remains prevalent, potentially hampering early haemostasis. AIM: Evaluate the dose-dependent efficacy, safety and immunogenicity of activated eptacog beta (rhFVIIa), a new recombinant inhibitor bypassing agent for the treatment of bleeding episodes (BEs)...
August 3, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28771891/successful-prophylaxis-using-activated-prothrombin-complex-concentrates-apcc-in-a-severe-haemophilia-a-patient-with-inhibitor-previously-unresponsive-to-on-demand-daily-infusions-of-apcc
#12
LETTER
S Furukawa, K Nogami, K Ogiwara, K Yada, M Shima
No abstract text is available yet for this article.
August 3, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28758324/surgery-and-survival-in-birth-cohorts-with-severe-haemophilia-and-differences-in-access-to-replacement-therapy-the-malm%C3%A3-experience
#13
M Osooli, K Steen Carlsson, J Astermark, E Berntorp
BACKGROUND: Persons with severe haemophilia require lifelong replacement therapy, prophylaxis, to prevent bleeding. Data describing long-term outcomes of prophylactic treatment are scarce. The aim of this study was to investigate joint surgery and survival among persons with severe haemophilia with special attention to access to prophylaxis in the early years of life. METHODS: Eligible participants had severe haemophilia A or B and were treated at the Malmö centre from the 1960s onward...
July 31, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28758308/concerns-regarding-clinical-trials-in-haemophilia-augmenting-iorio-s-research-and-policy-implications-of-a-recently-published-controlled-study-in-previously-untreated-haemophilia-patients-at-high-risk-of-inhibitor-development-haemophilia-2017-1-3-https-doi
#14
LETTER
https://www.readbyqxmd.com/read/28752639/perioperative-management-of-haemophilia-b-a-critical-appraisal-of-the-evidence-and-current-practices
#15
REVIEW
E J Neufeld, L Solimeno, D Quon, C Walsh, S Seremetis, D Cooper, N N Iyer, C S Hoxer, P Giangrande
BACKGROUND: While there is substantial literature addressing the principles of general management of haemophilia, literature on perioperative management of haemostasis is scarce. OBJECTIVE: The aim of this study was to better understand perioperative management among congenital haemophilia B patients (without inhibitors) and to gain insights into real-world surgical practices. METHOD: A systematic literature review, with an emphasis on haemophilia B, was conducted using EMBASE(®) , Medline(®) and the Cochrane Library...
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28752601/progression-of-hepatitis-c-in-the-haemophiliac-population-in-ireland-after-30-years-of-infection-in-the-pre-daa-treatment-era
#16
Niamh Murphy, Brian O'Mahony, Paula Flanagan, Declan Noone, Barry White, Colm Bergin, Suzanne Norris, Lelia Thornton
INTRODUCTION: Prior to the introduction of viral inactivation of factor concentrates and screening of blood, 225 people with haemophilia became infected with hepatitis C (HCV) in Ireland. AIM: Our aim was to assess liver disease progression and mortality in this population after 30 years of infection. METHODS: Demographic and clinical data were collected from medical records in five hepatology units and one infectious disease unit retrospectively in 2005, and on four subsequent occasions...
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750480/development-of-an-inhibitor-in-a-man-with-mild-haemophilia-a
#17
LETTER
M S Evans, M E Eyster
No abstract text is available yet for this article.
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750474/potential-misdiagnosis-of-von-willebrand-disease-and-haemophilia-caused-by-ineffective-mixing-of-thawed-plasma
#18
E J Favaloro, S Oliver, S Mohammed, M Ahuja, E Grzechnik, S Azimulla, J McDonald, G Lima-Oliveira, G Lippi
INTRODUCTION: von Willebrand disease (VWD) reflects a loss or dysfunction in von Willebrand factor (VWF), while haemophilia represents a loss or dysfunction of clotting factors such as factor VIII (FVIII) or FIX. Their diagnosis requires laboratory testing, with this potentially compromised by preanalytical events, including poor sample quality. This study assessed the effect of inadequate mixing as a potential cause of VWD and haemophilia misdiagnosis. METHODS: After completion of requested testing, 48 consecutive patient samples comprising separate aliquots from single collections were individually pooled, appropriately mixed, then frozen in separate aliquots, either at -20°C or -80°C for 2-7 days...
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750473/a-de-novo-factor-viii-mutation-in-a-haemophilia-b-family-leading-to-combined-deficiency-of-factor-viii-and-ix
#19
LETTER
A Prabhudesai, S Shanbhag, D Mirgal, N Kawankar, S Shetty
No abstract text is available yet for this article.
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750472/identifying-bleeding-go-with-the-flow
#20
M U Callaghan
No abstract text is available yet for this article.
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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