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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/29048712/efficacy-and-safety-of-nuwiq-%C3%A2-human-cl-rhfviii-in-patients-with-severe-haemophilia-a-undergoing-surgical-procedures
#1
N Zozulya, C M Kessler, A Klukowska, M von Depka, K Hampton, C R M Hay, M Jansen, J Bichler, S Knaub, S Rangarajan
INTRODUCTION: Haemophilia A patients are at a high risk of excess bleeding during surgeries. The aim of haemostatic therapy during the perioperative period is to normalize FVIII level perioperatively and postoperatively to maintain normal haemostasis until wound healing is complete. AIMS/METHODS: To examine the efficacy of Nuwiq(®) (simoctocog alfa, human-cl rhFVIII), a 4(th) generation recombinant FVIII produced in a human cell line, for surgical prophylaxis in patients with severe haemophilia A...
October 19, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29044825/pattern-of-bleeding-in-a-large-prospective-cohort-of-haemophilia-a-patients-a-three-year-follow-up-of-the-ahead-advate-in-haemophilia-a-outcome-database-study
#2
K Khair, M G Mazzucconi, R Parra, E Santagostino, D A Tsakiris, C Hermans, J Oldenburg, G Spotts, K Steinitz-Trost, A Gringeri
INTRODUCTION: Outcome data on treatment of patients with haemophilia A spanning several years of real-world evidence collection are currently very limited. AIM AND METHODS: The global prospective long-term Advate(®) Haemophilia A Outcome Database (AHEAD) cohort study collects real-world data from patients with severe and moderate haemophilia. We report an interim data read-out after three years of observation. RESULTS: A total of 522 patients were enrolled from 21 countries: 334 completed year 1 follow-up, 238 completed year 2 and 136 completed year 3, with an overall follow-up of 811 patient-years...
October 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28994494/development-of-a-thai-version-of-the-paediatric-bleeding-assessment-tool-thai-paediatric-bat-suitable-for-use-in-children-with-inherited-mucocutaneous-bleeding-disorders
#3
LETTER
S Pakdeeto, R Natesirinilkul, P Komwilaisak, M L Rand, V S Blanchette, S A Vallibhakara, N Sirachainan
No abstract text is available yet for this article.
October 10, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28984010/safety-and-dose-dependency-of-eptacog-beta-activated-in-a-dose-escalation-study-of-non-bleeding-congenital-haemophilia-a-or-b-patients-with-or-without-inhibitors
#4
J Ducore, J B Lawrence, M Simpson, L Boggio, A Bellon, J Burggraaf, J Stevens, M Moerland, J Frieling, J Reijers, M Wang
INTRODUCTION: Varying initial doses of activated eptacog beta (recombinant human FVIIa, rhFVIIa) may provide therapeutic options when treating bleeding in patients with congenital haemophilia who have developed inhibitory antibodies to factor VIII (FVIII) or factor IX (FIX). This study evaluated escalated doses of a new rhFVIIa product as a prelude to selecting the doses for clinical efficacy evaluation in haemophilia patients. AIM: To assess the safety, pharmacokinetics, and laboratory pharmacodynamics of 3 doses of rhFVIIa in non-bleeding patients with congenital haemophilia A or B with or without inhibitors...
October 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28976076/peripartum-bleeding-management-in-a-patient-with-caldag-gefi-deficiency
#5
LETTER
M Canault, P Saultier, S Fauré, M Poggi, A T Nurden, P Nurden, P E Morange, M-C Alessi, J-C Gris
No abstract text is available yet for this article.
October 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28960809/acquired-haemophilia-in-cancer-a-systematic-and-critical-literature-review
#6
REVIEW
M Napolitano, S Siragusa, S Mancuso, C M Kessler
AIM: There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer. METHODS: Treatment outcomes of AH were defined as complete response (CR), partial response (PR) or no response (NR), based on inhibitor eradication, coagulation factor VIII levels and bleeding control. Reported deaths were either related to cancer or bleeding...
September 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28960694/high-incidence-of-fxi-deficiency-in-a-spanish-town-caused-by-11-different-mutations-and-the-first-duplication-of-f11-results-from-the-yecla-study
#7
J Esteban, M E de la Morena-Barrio, S Salloum-Asfar, J Padilla, A Miñano, V Roldán, J M Soria, F Vidal, J Corral, V Vicente
INTRODUCTION: Factor XI (FXI) deficiency is a rare disorder with molecular heterogeneity in Caucasians but relatively frequent and molecularly homogeneous in certain populations. AIM: To characterize FXI deficiency in a Spanish town of 60 000 inhabitants. METHODS: A total of 324 764 APTT tests were screened during 20 years. FXI was evaluated by FXI:C and by Western blot. Genetic analysis of F11 was performed by sequencing, multiplex ligation-dependent probe amplification and genotyping...
September 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28949433/evaluation-of-the-self-administered-bleeding-assessment-tool-self-bat-in-haemophilia-carriers-and-correlations-with-quality-of-life
#8
LETTER
J E Young, J Grabell, A Tuttle, M Bowman, W M Hopman, D Good, N Rydz, J N Mahlangu, P D James
No abstract text is available yet for this article.
September 26, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28940852/anticoagulant-therapy-in-patients-with-glanzmann-s-thrombasthenia-is-it-possible
#9
LETTER
A Ferretti, C Santoro, G Ferrara, E Baldacci, F Barone, M G Mazzucconi
No abstract text is available yet for this article.
September 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28925586/haemopref-further-evaluation-of-patient-perception-and-preference-for-treatment-in-a-real-world-setting
#10
S Bonanad, M Schulz, A Gordo, D Spurden, M Cicchetti, J C Cappelleri, C Tolley, H Staunton, E Brohan
INTRODUCTION: Adherence to haemophilia A (HA) treatment may be influenced by patients' beliefs about their condition and treatment. Furthermore, difficulties administering treatment may lead to poor adherence. New treatment strategies aim to reduce the burden associated with administration and to improve patient perception of treatment, which, in turn, increase adherence levels. AIMS: This study aimed to examine patient perception of HA treatment and related factors using patient-reported outcome (PRO) questionnaires and to confirm the psychometric properties of a recently developed questionnaire, the HaemoPREF...
September 19, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28925572/tracheal-stenosis-in-a-paediatric-patient-with-severe-haemophilia-a-and-fviii-inhibitors
#11
LETTER
H Pergantou, Z Zachou, K Vachlas, A Dettoraki, L Lykopoulou, K Parpounas, H Platokouki
No abstract text is available yet for this article.
September 19, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28922525/describing-the-quality-of-life-of-boys-with-haemophilia-in-china-results-of-a-multicentre-study-using-the-cho-klat
#12
L Tang, W Xu, C G Li, F Hou, X Q Feng, H Wang, X J Li, W L Li, J P Liu, L R Sun, S H Wang, J Jin, Q Fang, K H Luke, M C Poon, V S Blanchette, K Usuba, N L Young, R Wu
INTRODUCTION: The treatment of haemophilia varies across countries and across regions within some countries. Similar variation has been observed in health-related quality of life (HR-QoL). Relatively little is known about the HR-QoL of boys with haemophilia in China. AIM: The aim of this study was to describe the HR-QoL of boys with haemophilia in China using the Canadian Haemophilia Outcomes-Kids Life Assessment Tool (CHO-KLAT). METHODS: Boys (4-18 years of age) with haemophilia and their parents were enroled in a cross-sectional study...
September 18, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28922522/chromogenic-analysis-of-fix-activity-in-haemophilia-b-patients-treated-with-nonacog-beta-pegol
#13
LETTER
G Young, M Ezban, W H O Clausen, C Negrier, J Oldenburg, M Shima
No abstract text is available yet for this article.
September 18, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28906056/perioperative-management-of-haemophilia-a-using-recombinant-factor-viii-fc-fusion-protein-in-a-patient-undergoing-endoscopic-nasal-pituitary-adenomectomy-for-a-growth-hormone-producing-pituitary-adenoma
#14
LETTER
A Okamoto, K Yamamoto, G Eguchi, T Yamaguchi, N Nishi, T Yamada, E Chen, Y Maeda
No abstract text is available yet for this article.
September 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28891593/effect-of-radiosynoviorthesis-on-the-progression-of-arthropathy-and-haemarthrosis-reduction-in-haemophilic-patients
#15
M Querol-Giner, S Pérez-Alenda, M Aguilar-Rodríguez, J J Carrasco, S Bonanad, F Querol
INTRODUCTION: Repeated haemarthrosis is widely accepted as the triggering cause of synovitis and haemophilic arthropathy. A first-line treatment of chronic synovitis is radiosynoviorthesis (RS). The aim of this study was to evaluate the RS effects on the progression of arthropathy and on a reduction in bleeding in patients with haemophilia. METHODS: An observational-retrospective study was performed. Bleeding episodes in the 12 months following and in the 12 months preceding RS was compared...
September 11, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28891589/association-of-factor-viii-and-factor-ix-mutations-hla-class-ii-tumour-necrosis-factor-%C3%AE-and-interleukin-10-on-inhibitor-development-among-thai-haemophilia-a-and-b-patients
#16
LETTER
A Chuansumrit, W Sasanakul, N Sirachainan, P Kadegasem, P Wongwerawattanakoon, L Mahaklan, O Nathalang
No abstract text is available yet for this article.
September 11, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28873289/monitoring-joint-health-in-haemophilia-factors-associated-with-deterioration
#17
I A R Kuijlaars, M A Timmer, P de Kleijn, M F Pisters, K Fischer
INTRODUCTION: Joint bleeds in patients with haemophilia may result in haemophilic arthropathy. Monitoring joint health is essential for identifying early signs of deterioration and allows timely adjustment of treatment. AIM: The aim was to describe changes in joint health over 5-10 years follow-up and identify factors associated with joint health deterioration in patients with haemophilia. METHODS: A post hoc analysis was performed from previous cohort studies in patients with moderate/severe haemophilia, ≥16 years...
September 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28873279/differences-in-bleeding-phenotype-and-provider-interventions-in-postmenarchal-adolescents-when-compared-to-adult-women-with-bleeding-disorders-and-heavy-menstrual-bleeding
#18
L V Srivaths, Q C Zhang, V R Byams, J E Dietrich, A H James, P A Kouides, R Kulkarni
INTRODUCTION: Due to lack of patient/health care provider awareness causing delayed diagnosis, the bleeding phenotype and provider interventions in adolescents with heavy menstrual bleeding (HMB) and bleeding disorders (BD) may be different when compared to adults. AIM: The aim of this study was to compare/characterize bleeding phenotype and provider interventions in postmenarchal adolescents < 18 years and premenopausal adults ≥ 18 years with HMB and BD...
September 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28873263/continuous-infusion-of-coagulation-factor-concentrates-during-intensive-treatment
#19
REVIEW
P A Holme, G E Tjønnfjord, A Batorova
In clinical management of bleeds and surgical procedures in patients suffering from bleeding disorders either repetitive bolus injections (BI) or continuous infusion (CI) can be used for coagulation factor replacement. Continuous infusion seems to be an attractive route of administration and may be considered if replacement therapy is required for more than 3 days. The strongest argument favouring continuous infusion is its superiority in providing the patient with a safe and constant level of the deficient coagulation factor by balancing input with clearance...
September 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28869681/surgical-treatment-of-a-giant-iliopsoas-haemophilic-pseudotumour-with-adjacent-structure-compressions-a-case-report
#20
LETTER
Y Y Bian, H Wu, Z Huang, J Zhai, Y Liu, X S Weng
No abstract text is available yet for this article.
September 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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