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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/28544490/evaluation-of-outcomes-of-suction-drainage-in-patients-with-haemophilic-arthropathy-undergoing-total-knee-arthroplasty
#1
S M J Mortazavi, M A Firoozabadi, A Najafi, P Mansouri
INTRODUCTION: Closed suction drainage has been extensively applied for orthopaedic procedures for the sake of avoiding fluid collections around the wound at the operated limb. AIM: We believed that without application of suction drainage after total knee arthroplasty (TKA), the risk of infection and haematoma formation will not increase while the need for blood transfusion because of decreased blood loss will be lesser. METHODS: In a prospective randomized clinical trial from April 2010 to April 2014, 176 patients with haemophilia who underwent TKA were included...
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544483/polypharmacy-in-older-adults-with-severe-haemophilia
#2
LETTER
S Riva, M E Mancuso, L Cortesi, A Nobili, E Santagostino, F Peyvandi, P M Mannucci
No abstract text is available yet for this article.
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544271/radionuclide-synovectomy-synoviorthesis-rs-in-persons-with-bleeding-disorders-a-review-of-impact-of-national-guidance-on-frequency-of-rs-using-the-athndataset
#3
LETTER
R Sharma, A Dunn, D Aschman, D Cheng, A Wheeler, A Soni, C McGuinn, C Knoll, D T Stein, G Young, J French, J Sanders, J A Davis, M Tarantino, M Lim, R Gruppo, R Sidonio, S Ahuja, S Carpenter, S Pipe, A Shapiro
No abstract text is available yet for this article.
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544263/impact-of-a-product-specific-reference-standard-for-the-measurement-of-a-pegylated-rfviii-activity-the-swiss-multicentre-field-study
#4
O Bulla, A Poncet, L Alberio, L M Asmis, A Gähler, L Graf, M Nagler, J-D Studt, D A Tsakiris, P Fontana
INTRODUCTION: Measuring factor VIII (FVIII) activity can be challenging when it has been modified, such as when FVIII is pegylated to increase its circulating half-life. Use of a product-specific reference standard may help avoid this issue. AIM: Evaluate the impact of using a product-specific reference standard for measuring the FVIII activity of BAX 855 - a pegylated FVIII - in eight of Switzerland's main laboratories. METHODS: Factor VIII-deficient plasma, spiked with five different concentrations of BAX 855, plus a control FVIII sample, was sent to the participating laboratories...
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544163/the-impact-of-clinical-practice-on-the-outcome-of-central-venous-access-devices-in-children-with-haemophilia
#5
K Khair, S Ranta, A Thomas, K Lindvall
INTRODUCTION: Central venous access devices facilitate home treatment in boys with haemophilia. These are usually fully implanted lines, referred to as ports. Caregivers are taught to manage the port using sterile techniques and maintaining patency by flushing with saline or heparin solution. National and international guidelines for the home care of ports are lacking. AIM: To evaluate if infection or occlusion rates differ between home care regimens used for ports in children with haemophilia...
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544125/mobile-health-services-in-haemophilia-opportunities-and-risks
#6
LETTER
A-B Del Río-Lanza, A Suárez-Vázquez, L Suárez-Álvarez, V Iglesias-Argüelles
No abstract text is available yet for this article.
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544055/low-dose-prophylaxis-for-children-with-haemophilia-in-a-resource-limited-setting-in-south-india-a-clinical-audit-report
#7
LETTER
N Sidharthan, R Sudevan, V Narayana Pillai, S Mathew, M Raj, D Viswam, C Joseph, A Sudhakar
No abstract text is available yet for this article.
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544023/from-treatment-to-prevention-of-bleeds-what-more-evidence-do-we-need
#8
H M van den Berg
No abstract text is available yet for this article.
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28544019/let-s-talk-period-preliminary-results-of-an-online-bleeding-awareness-knowledge-translation-project-and-bleeding-assessment-tool-promoted-on-social-media
#9
E Reynen, J Grabell, A K Ellis, P James
INTRODUCTION: Undiagnosed bleeding disorders are common and can pose significant health risks, especially for women. Recently, a self-administered bleeding assessment tool (Self-BAT) was validated in von Willebrand disease. AIM: To increase awareness of undiagnosed bleeding disorders through the use of an informational website (http://letstalkperiod.ca) targeted at women in their reproductive years. METHODS: The Let's Talk Period website was built in consultation with a medical communications company and focus groups of women, with the aim of clearly presenting key messages around menstrual bleeding...
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28543946/predicting-dose-sparing-benefit-and-bleeding-risk-of-pharmacokinetic-based-personalized-prophylactic-dosing-of-factor-viii-products
#10
M A Tegenge, H Yang, R A Forshee
BACKGROUND: Previously published factor VIII (FVIII) pharmacokinetic (PK)-based dosing approaches employ fixed infusion interval with a wide dose range that may lead to increased risk of bleeding, excessive doses or decreased health-related quality of life. AIM: The objectives of the study includes (i) personalizing infusion interval in lieu of fixed infusion, (ii) constraining dose within the range of 10-50 IU/kg and (iii) characterizing bleeding risk of PK-based dosing in comparison with empiric standard doses...
May 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28497894/sensor-based-gait-analysis-as-a-simple-tool-to-measure-gait-in-haemophilia-patients
#11
LETTER
S C M Stoof, D Hodgins, F W G Leebeek, M H Cnossen, H L D Horemans, S F E Praet, M J H A Kruip
No abstract text is available yet for this article.
May 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28497886/acquired-von-willebrand-syndrome-in-haematologic-malignancies-how-the-clinical-laboratory-correlation-improves-a-challenging-diagnosis-a-case-series
#12
LETTER
T Fidalgo, G Ferreira, A C Oliveira, C Silva Pinto, P Martinho, M J Mendes, M Duarte, R Salvado, M L Ribeiro
No abstract text is available yet for this article.
May 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28497870/issues-in-reproductive-health-in-females-having-inherited-bleeding-disorders-in-pakistan
#13
LETTER
S Hussain, B Moiz, S Aqeel, N Zaidi
No abstract text is available yet for this article.
May 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28497866/evaluation-of-a-new-commercial-von-willebrand-factor-multimer-assay
#14
LETTER
E J Favaloro, S Oliver
No abstract text is available yet for this article.
May 12, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28475272/recombinant-factor-viia-addition-to-haemophilic-blood-perfused-over-collagen-tissue-factor-can-sufficiently-bypass-the-factor-ixa-viiia-defect-to-rescue-fibrin-generation
#15
R Li, K A Panckeri, P F Fogarty, A Cuker, S L Diamond
INTRODUCTION: Factor VIII (FVIII) or factor IX (FIX)-deficient haemophilic patients display deficits in platelet and fibrin deposition under flow detectable in microfluidics. Compared to fibrin generation, decreased platelet deposition in haemophilic blood flow is more easily rescued with recombinant factor VIIa (rFVIIa), whereas rFVIIa requires FXIIa participation to generate fibrin when tissue factor (TF) is absent. AIMS: Perfusion of haemophilic whole blood (WB) over collagen/TF surfaces was used to determine whether rFVIIa/TF was sufficient to bypass poor FIXa/FVIIIa function in blood from patients with haemophilia A and B...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28475226/molecular-cytogenetic-characterization-of-five-f8-complex-rearrangements-utility-for-haemophilia-a-genetic-counselling
#16
Y Jourdy, N Chatron, M Fretigny, M L Carage, H Chambost, S Claeyssens-Donadel, V Roussel-Robert, C Negrier, D Sanlaville, C Vinciguerra
BACKGROUND: Genomic inversions are usually balanced, but unusual patterns have been described in haemophilia A (HA) patients for intron 22 (Inv22) and intron 1 (Inv1) inversions leading to the hypothesis of more complex rearrangements involving deletions or duplications. AIM: To characterize five abnormal patterns either in Southern blot and long-range PCR for Inv22 or in PCR for Inv1. MATERIALS AND METHODS: All patients were studied using cytogenetic microarray analysis (CMA)...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28474428/successful-management-of-refractory-haematuria-with-recombinant-porcine-factor-viii-replacement-for-paediatric-congenital-haemophilia-a-with-high-titre-inhibitor
#17
LETTER
S Jain, S Gupta, S J Ambrusko, A Shapiro
No abstract text is available yet for this article.
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470911/platelet-rich-plasma-for-chronic-synovitis-treatment-in-patients-with-haemophilia
#18
H Caviglia, M E Landro, C Daffunchio, G Galatro, A L Douglas Price, P Salgado, D Neme
INTRODUCTION: Haemophilic synovitis is caused by chronic accumulation of blood in the joint. Conservative treatment is insufficient to solve this pathology. Platelet-rich plasma (PRP) has a high concentration of growth factors (GFs) that play a key role in regulation and stimulation of healing processes. The aim of this study was to describe the effect of PRP injection in chronic synovitis of the joints in patients with haemophilia (PWH). PATIENTS AND METHODS: Nineteen patients with 28 joints were treated at our centre in Buenos Aires, Argentina between December 2014 and December 2015...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470862/first-report-on-the-safety-and-efficacy-of-an-extended-half-life-glycopegylated-recombinant-fviii-for-major-surgery-in-severe-haemophilia-a
#19
K Hampton, P Chowdary, S Dunkley, S Ehrenforth, L Jacobsen, A Neff, E Santagostino, J Sathar, H Takedani, C M Takemoto, C Négrier
BACKGROUND: N8-GP (turoctocog alfa pegol) is an extended half-life glycoPEGylated recombinant factor VIII (FVIII) product developed for the prevention and treatment of bleeds in haemophilia A patients. AIM: This is a planned interim analysis of pathfinder™3, an international, open-label, Phase 3 trial evaluating the efficacy and safety (including immunogenicity) of N8-GP administered before, during and after major surgery in severe haemophilia A patients aged ≥12 years...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470853/role-of-red-blood-cells-in-the-anemia-associated-bleeding-under-high-shear-conditions
#20
H Yaoi, Y Shida, K Ogiwara, K Hosokawa, M Shima, K Nogami
BACKGROUND: Red blood cells (RBCs) contribute to hemostasis under blood-flow, and anemia might contribute to a hemorrhagic diathesis. The majority of current laboratory techniques to assess hemostasis do not consider the effects of RBCs. An assay to determine the role of RBCs in hemostasis could be beneficial for clinical management. OBJECTIVES: To investigate the influence of RBCs in hemostasis. METHODS: Hemostasis was investigated using a novel microchip flow-chamber system (T-TAS(®) ) in an anemic patient with von Willebrand disease...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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