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Haemophilia

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https://www.readbyqxmd.com/read/28636264/desmopressin-in-haemophilia-the-need-for-a-standardised-clinical-response-and-individualised-test-regimen
#1
S C M Stoof, L M Schütte, F W G Leebeek, M H Cnossen, M J H A Kruip
INTRODUCTION: Due to interindividual variation in desmopressin response, non-severe haemophilia A patients require desmopressin testing prior to therapeutic treatment. However, adequate response or frequency of blood sampling is not standardised in international guidelines. Consequently, various definitions and blood sampling protocols are currently applied. Interestingly, sustainability of desmopressin response is not incorporated into these definitions. AIM: To study desmopressin response rates in a cohort of non-severe haemophilia A patients using currently accepted desmopressin response definitions...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28636084/co-morbidities-and-bleeding-in-elderly-patients-with-haemophilia-a-survey-of-the-german-austrian-and-swiss-society-of-thrombosis-and-haemostasis-research-gth
#2
W Miesbach, S-E Reitter-Pfoertner, R Klamroth, F Langer, H-H Wolf, A Tiede, B Siegmund, U Scholz, P R Müller, H Eichler, I Pabinger
BACKGROUND: Nowadays patients with haemophilia survive longer due to improvements in haemophilia care. It has been hypothesized that the bleeding type and frequency may vary with age and are influenced by co-morbidities and co-medication in elderly patients. OBJECTIVES: To investigate a large group of patients older than 60 years of age with haemophilia concerning haemophilia treatment, bleeding pattern changes, co-morbidities, co-medication, bleeding sites and patient mortality...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28636076/prevalence-and-impact-of-obesity-in-people-with-haemophilia-review-of-literature-and-expert-discussion-around-implementing-weight-management-guidelines
#3
REVIEW
S Kahan, A Cuker, R F Kushner, J Maahs, M Recht, T Wadden, T Willis, S Majumdar, D Ungar, D Cooper
Obesity affects more than 35% of Americans, increasing the risk of more than 200 comorbid conditions, impaired quality of life and premature mortality. This review aimed to summarize literature published over the past 15 years regarding the prevalence and impact of obesity in people with haemophilia (PWH) and to discuss implementing general guidelines for weight management in the context of the haemophilia comprehensive care team. Although few studies have assessed the effects of obesity on haemophilia-specific outcomes, existing evidence indicates an important impact of weight status on lower extremity joint range of motion and functional disability, with potentially important effects on overall quality of life...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28621454/adult-males-with-haemophilia-have-a-different-macrovascular-and-microvascular-endothelial-function-profile-compared-with-healthy-controls
#4
H Sun, M Yang, M Fung, S Chan, M Jawi, T Anderson, M-C Poon, S Jackson
INTRODUCTION: Endothelial function has been identified as an independent predictor of cardiovascular risk in the general population. It is unclear if the haemophilia population has a different endothelial function profile compared to the healthy population. AIM: This prospective study aims to assess if there is a difference in endothelial function between haemophilia patients and healthy controls, and the impact of endothelial function on vascular outcomes in the haemophilia population...
June 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28594467/biochemical-characterization-of-lr769-a-new-recombinant-factor-viia-bypassing-agent-produced-in-the-milk-of-transgenic-rabbits
#5
G Chevreux, N Tilly, Y Leblanc, C Ramon, V Faid, M Martin, F Dhainaut, N Bihoreau
BACKGROUND: The bypassing agent factor VII (FVIIa) is a first-line therapy for the treatment of acute bleeding episodes in patients with haemophilia and high-titre inhibitors. FVIIa is a highly post-translationally modified protein that requires eukaryotic expression systems to produce a fully active molecule. A recombinant FVIIa was produced in the milk of transgenic rabbits to increase expression and provide an efficient, safe and affordable product after purification to homogeneity (LR769)...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28594458/concizumab-an-anti-tissue-factor-pathway-inhibitor-antibody-induces-increased-thrombin-generation-in-plasma-from-haemophilia-patients-and-healthy-subjects-measured-by-the-thrombin-generation-assay
#6
E K Waters, J Sigh, U Friedrich, I Hilden, B B Sørensen
AIMS: Concizumab, a humanized monoclonal antibody against tissue factor pathway inhibitor (TFPI), is being developed as a subcutaneously (s.c.) administered treatment for haemophilia. It demonstrated a concentration-dependent procoagulant effect in functional TFPI assays; however, global haemostatic assays, such as the thrombin generation assay (TGA), offer a more complete picture of coagulation. We investigated how concizumab affects thrombin generation following ex vivo spiking in plasma from haemophilia patients using the TGA, and if the assay can detect the effect of multiple s...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28594432/involvement-of-the-ige-basophil-system-and-mild-complement-activation-in-haemophilia-b-with-anti-factor-ix-neutralizing-antibodies-and-anaphylaxis
#7
M Cugno, M E Mancuso, A Tedeschi, E Santagostino, M Lorini, V Carbonelli, F Peyvandi, P M Mannucci
INTRODUCTION: Patients with haemophilia B who develop factor IX (FIX) neutralizing antibodies (inhibitors) after FIX infusion are at high risk of hypersensitivity reactions upon FIX re-exposure, but the underlying mechanisms are incompletely understood. AIM: To investigate biomechanisms of FIX hypersensitivity. METHODS: A cellular antigen stimulation test (CAST) was employed to evaluate leukotriene C4 (LTC4) release from basophils stimulated by FIX in three treated children with haemophilia B, one of whom developed FIX inhibitor and experienced anaphylaxis following FIX re-exposure...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574229/the-effects-of-joint-disease-inhibitors-and-other-complications-on-health-related-quality-of-life-among-males-with-severe-haemophilia-a-in-the-united-states
#8
J M Soucie, S D Grosse, A-E-A Siddiqi, V Byams, J Thierry, M M Zack, A Shapiro, N Duncan
INTRODUCTION: Health-related quality of life (HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference-based HRQoL measures are needed to model the cost-effectiveness of prevention strategies. AIM: We examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference-based measures of HRQoL...
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574216/imaging-of-haemophilic-arthropathy-in-growing-joints-pitfalls-in-ultrasound-and-mri
#9
REVIEW
M Soliman, P Daruge, S S J Dertkigil, E De Avila Fernandes, J R Negrao, S de Aguiar Vilela Mitraud, E T I Sakuma, A R C Fernandes, N Zhang, A Huo, Y-J Li, F Zhou, B M Rodrigues, A Mohanta, V S Blanchette, A S Doria
The purpose of this review was to summarize the current knowledge on the utilization of magnetic resonance imaging (MRI) and ultrasound (US) for assessing arthropathy in children and adolescents with haemophilia and to recognize the limitations of each imaging modality and pitfalls in the diagnosis of soft tissue and osteochondral abnormalities. Awareness of MRI and US limitations and pitfalls in the assessment of joints in persons with haemophilia is essential for accurate diagnosis and optimal management of haemophilic arthropathy...
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574215/haemophilia-care-in-europe-a-survey-of-37-countries
#10
B O Mahony, L Savini, J O Hara, A Bok
INTRODUCTION: The European Haemophilia Consortium (EHC) is an international non-profit organization representing 45 national patients' organizations in Europe. Every 3 years, the EHC circulates a survey to its national member organizations to assess the state of haemophilia care. AIM: The purpose of this exercise is to ascertain information about the organization of haemophilia care and treatment availability at national levels. Furthermore, the survey provides a basis from which the EHC are able to monitor the unmet need and stability of care/treatment access in the individual member countries...
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574205/first-line-immune-tolerance-induction-for-children-with-severe-haemophilia-a-a-protocol-from-the-uk-haemophilia-centre-doctors-organisation-inhibitor-and-paediatric-working-parties
#11
P Collins, E Chalmers, J Alamelu, C Hay, R Liesner, M Makris, M Mathias, J Payne, S Rangarajan, M Richards, K Talks, O Tunstall, M Williams, D P Hart
No abstract text is available yet for this article.
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574191/point-of-care-ultrasound-in-haemophilia-building-a-strong-foundation-for-clinical-implementation
#12
W Lawson, M Uy, K Strike, A Iorio, N Stein, L Koziol, A Chan
No abstract text is available yet for this article.
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574179/episodic-replacement-of-clotting-factor-concentrates-does-not-prevent-bleeding-or-musculoskeletal-damage-the-musfih-study
#13
P Poonnoose, J D A Carneiro, A L Cruickshank, M El Ekiaby, R P Perez Bianco, M C Ozelo, N De Bosch, M Baghaipour, S L Tien, A Chuansumrit, E A D'Amico, A van Zyl, A Sabour, M Candela, J B S Ricciardi, A Ruiz-Sàez, R Ravanbod, J C L Lam, S Jaovisidha, M L Kavitha, S Gibikote, N Shyamkumar, A Srivastava
PATIENTS AND METHODS: A longitudinal study was carried out in 255 children from 10 centres in nine developing countries over 5 years to assess the musculoskeletal outcome of children on episodic factor replacement. Outcome was documented by assessment of the annual joint bleeding rate (AJBR), WFH clinical and Pettersson radiological joint scores as well as the FISH score for activities. Of the 203 patients for whom data was available at the end of 5 years, 164 who had received only episodic treatment are included in this report...
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28574162/real-world-resource-use-and-costs-of-haemophilia-a-related-bleeding
#14
A Shrestha, A Eldar-Lissai, N Hou, D N Lakdawalla, K Batt
INTRODUCTION: Prophylaxis treatment is recommended for haemophilia patients, but associated real-world economic costs and potential cost-savings associated with improved disease management are not fully known. This study aimed to assess haemophilia A-related resource use and cost by treatment type (prophylaxis versus non-prophylaxis) and any associated cost-savings. METHODS: Truven MarketScan Commercial claims data (2004-2012) were used to identify haemophilia A-related healthcare utilization, healthcare costs and patterns of prophylaxis and non-prophylaxis treatment among 6- to 64-year-old males...
June 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28569181/the-cost-of-severe-haemophilia-in-europe-the-chess-study
#15
Jamie O'Hara, David Hughes, Charlotte Camp, Tom Burke, Liz Carroll, Daniel-Anibal Garcia Diego
BACKGROUND: Severe haemophilia is associated with major psychological and economic burden for patients, caregivers, and the wider health care system. This burden has been quantified and documented for a number of European countries in recent years. However, few studies have taken a standardised methodology across multiple countries simultaneously, and sought to amalgamate all three levels of burden for severe disease. The overall aim of the 'Cost of Haemophilia in Europe: a Socioeconomic Survey' (CHESS) study was to capture the annualised economic and psychosocial burden of severe haemophilia in five European countries...
May 31, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28567541/gene-therapy-for-monogenic-liver-diseases-clinical-successes-current-challenges-and-future-prospects
#16
Julien Baruteau, Simon N Waddington, Ian E Alexander, Paul Gissen
Over the last decade, pioneering liver-directed gene therapy trials for haemophilia B have achieved sustained clinical improvement after a single systemic injection of adeno-associated virus (AAV) derived vectors encoding the human factor IX cDNA. These trials demonstrate the potential of AAV technology to provide long-lasting clinical benefit in the treatment of monogenic liver disorders. Indeed, with more than ten ongoing or planned clinical trials for haemophilia A and B and dozens of trials planned for other inherited genetic/metabolic liver diseases, clinical translation is expanding rapidly...
May 31, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28547182/three-year-survival-of-art-high-viscosity-glass-ionomer-and-resin-composite-restorations-in-people-with-disability
#17
Gustavo F Molina, Denise Faulks, Ignacio Mazzola, Ricardo J Cabral, Jan Mulder, Jo E Frencken
OBJECTIVES: To assess the 3-year cumulative survival rate of atraumatic restorative treatment (ART) and conventional resin composite restorations (CRT) placed in persons with disability. MATERIALS AND METHODS: Patients referred for restorative care to the Haemophilia Foundation special care service were treated by one of two specialists. Patients and/or caregivers were provided with written and verbal information regarding treatment options and selected the alternative they preferred...
May 25, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28544490/evaluation-of-outcomes-of-suction-drainage-in-patients-with-haemophilic-arthropathy-undergoing-total-knee-arthroplasty
#18
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/28544163/the-impact-of-clinical-practice-on-the-outcome-of-central-venous-access-devices-in-children-with-haemophilia
#19
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/28520202/haemophilia-treatment-for-all-and-the-role-of-tolerance-difference-and-education
#20
EDITORIAL
Alain Weill
No abstract text is available yet for this article.
May 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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