keyword
MENU ▼
Read by QxMD icon Read
search

Haemophilia

keyword
https://www.readbyqxmd.com/read/28944952/bypassing-agent-prophylaxis-in-people-with-hemophilia-a-or-b-with-inhibitors
#1
REVIEW
Chatree Chai-Adisaksopha, Sarah J Nevitt, Mindy L Simpson, Maissaa Janbain, Barbara A Konkle
BACKGROUND: People with hemophilia A or B with inhibitors are at high risk of bleeding complications. Infusion of bypassing agents, such as recombinant activated FVII (rFVIIa) and plasma-derived activated prothrombin complex concentrate, are suggested as alternative therapies to factor VIII (haemophilia A) or IX (haemophilia B) for individuals who no longer respond to these treatments because they develop inhibitory antibodies. The ultimate goal of treatment is to preserve the individual's joints, otherwise destroyed by recurrent bleeds...
September 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28943040/treatment-of-musculo-skeletal-pain-in-haemophilia
#2
REVIEW
E Carlos Rodriguez-Merchan
Musculo-skeletal pain treatment is inadequate in many haemophilic patients. Analgesics are used only by 36% of adult patients. FVIII/FIX intravenous infusion is mainly used to lessen pain, followed in frequency by usage of NSAIDS (primarily COX-2 inhibitors). In about 30% of patients, pain continues after infusion of F VIII/IX. In acute haemarthroses pain treatment must continue until total disappearance (checked by ultrasonography) and include haematologic treatment, short-term rest of the involved joint, cryotherapy, joint aspiration and analgesic medication (paracetamol in mild pain, metamizole for more intense pain, and in a few precise patients, soft opioids such as codeine or tramadol)...
September 19, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28942686/fibrin-glue-for-local-haemostasis-in-haemophilia-surgery
#3
E Carlos Rodriguez-Merchan
INTRODUCTION: Local fibrin glue (FG) appears to be a useful local haemostatic agent for severe haemorrhage in people with haemophilia (PWH) undergoing surgical procedures. AIM: To evaluate the role of local FG in PWH. METHODS: A review of the literature on the topic has been performed. RESULTS: Local FG is not always necessary to achieve haemostasis in all surgical procedures performed in PWH. However, it could be a good adjunct therapy, primarily when a surgical field will bleed more than expected (e...
September 23, 2017: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/28925586/haemopref-further-evaluation-of-patient-perception-and-preference-for-treatment-in-a-real-world-setting
#4
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/28924993/acquired-a-haemophilia-a-case-report
#5
E Cinotti, E Trovato, M Fimiani, P Rubegni
Acquired haemophilia A (AHA) due to autoantibodies against Factor VIII (FVIII) is a rare disease associated with severe bleeding. Since diffuse cutaneous hematomas and ecchymoses are the main clinical signs of this condition, dermatologists should be aware about this disease. Here, we present a case of AHA in a patient with systemic lupus erythematosus (SLE). This article is protected by copyright. All rights reserved.
September 19, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
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
#6
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/28921738/partnering-to-change-the-world-for-people-with-haemophilia-7th-haemophilia-global-summit-madrid-spain-22-24-september-2016
#7
Gerry Dolan
The 7th Haemophilia Global Summit was held in Madrid, Spain, in September 2016. With a programme designed, for the 6th consecutive year, by a Scientific Steering Committee of haemophilia experts, the aim of the summit was to share optimal management strategies for haemophilia at all life stages and to provide an opportunity for specialists from across the haemophilia multidisciplinary care team to engage in discussion and debate with leading international experts on current and future areas of research. Topics covered ranged from the optimisation of haemophilia management, emerging issues in clinical care, practical approaches and future perspectives, in addition to patient engagement and empowerment in modern haemophilia care...
October 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28921737/partnering-to-change-the-world-for-people-with-haemophilia-7th-haemophilia-global-summit-madrid-spain-22-24-september-2016
#8
(no author information available yet)
No abstract text is available yet for this article.
October 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28920578/participation-in-physical-and-sportive-activities-among-adult-turkish-people-with-haemophilia-a-single-centre-experience
#9
Arni Lehmeier, Muhlis Cem Ar, Sevil Sadri, Mehmet Yürüyen, Zafer Başlar
No abstract text is available yet for this article.
September 18, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28900904/lonoctocog-alfa-a-review-in-haemophilia-a
#10
Zaina T Al-Salama, Lesley J Scott
Lonoctocog alfa (rVIII-SingleChain; Afstyla(®)) is a novel single-chain recombinant factor VIII (FVIII) molecule, with a truncated B-domain and the heavy and light chains covalently linked to form a stable and homogenous drug that binds with high affinity to von Willebrand factor (VWF). Intravenous lonoctocog alfa is approved for the prophylaxis and treatment of bleeding in patients with haemophilia A in several countries worldwide. In two pivotal, multicentre trials, lonoctocog alfa was effective in the treatment of bleeding episodes and as prophylaxis, including for perioperative management in adults, adolescents and children...
September 12, 2017: Drugs
https://www.readbyqxmd.com/read/28891593/effect-of-radiosynoviorthesis-on-the-progression-of-arthropathy-and-haemarthrosis-reduction-in-haemophilic-patients
#11
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/28876509/clot-waveform-analysis-where-do-we-stand-in-2017
#12
REVIEW
P O Sevenet, F Depasse
Analysis of the optical waveform generated during global coagulation assays, such as activated partial thromboplastin time and prothrombin time, can provide much precious information on the global coagulation state of the plasma sample tested, in addition to a single clotting time. Many studies have been published concerning patient diagnosis and management in haemophilia A, and in the early diagnosis and prognosis of disseminated intravascular coagulation and sepsis. However, many other works have also been published on further potential clinical applications such as lupus anticoagulant diagnosis and anticoagulant monitoring...
September 6, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28873289/monitoring-joint-health-in-haemophilia-factors-associated-with-deterioration
#13
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/28873263/continuous-infusion-of-coagulation-factor-concentrates-during-intensive-treatment
#14
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/28871021/effectiveness-of-two-psychological-interventions-for-pain-management-emotional-regulation-and-promotion-of-quality-of-life-among-adult-portuguese-men-with-haemophilia-psy-haemopeq-study-protocol-for-a-single-centre-prospective-randomised-controlled-trial
#15
Patrícia Ribeiro Pinto, Ana Cristina Paredes, Patrício Costa, Manuela Carvalho, Manuela Lopes, Susana Fernandes, Susana Pedras, Armando Almeida
INTRODUCTION: Haemophilia is a bleeding disorder associated with significant pain, emotional distress, quality of life (QoL) impairment and considerable healthcare costs. Psychosocial health and effective pain management are considered essential end points for optimal haemophilia care, but there is a significant gap in evidence-based treatments targeting these outcomes in people with haemophilia (PWH). Psychological interventions are cost-effective in promoting emotional well-being, QoL and pain control, although these have been scarcely used in haemophilia field...
September 3, 2017: BMJ Open
https://www.readbyqxmd.com/read/28863796/a-promising-new-treatment-horizon-for-haemophilia-a
#16
EDITORIAL
The Lancet Haematology
No abstract text is available yet for this article.
September 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28838029/radionuclide-synovectomy-synoviorthesis-rs-in-patients-with-bleeding-disorders-a-review-of-patient-and-procedure-demographics-and-functional-outcomes-in-the-athndataset
#17
C McGuinn, D Cheng, D Aschman, S L Carpenter, R Sidonio, A Soni, M D Tarantino, A P Wheeler, A L Dunn
INTRODUCTION: Radionuclide synovectomy/synoviorthesis (RS) to manage proliferative synovitis in persons with bleeding disorders has been utilized for decades; however, aggregate US results are limited. AIM: To determine the prevalence of RS utilization, patient and procedure related demographics and functional outcomes in United States haemophilia treatment centres (HTCs). The ATHNdataset includes US patients with bleeding disorders who have authorized the sharing of their demographic and clinical information for research...
August 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28837614/mice-deficient-in-the-anti-haemophilic-coagulation-factor-viii-show-increased-von-willebrand-factor-plasma-levels
#18
Klytaimnistra Kiouptsi, Alexandra Grill, Amrit Mann, Mareike Döhrmann, Maren Lillich, Sven Jäckel, Frano Malinarich, Henning Formes, Davit Manukyan, Saravanan Subramaniam, Avinash Khandagale, Cornelia Karwot, Serge C Thal, Markus Bosmann, Inge Scharrer, Kerstin Jurk, Christoph Reinhardt
Von Willebrand factor (VWF) is the carrier protein of the anti-haemophilic Factor VIII (FVIII) in plasma. It has been reported that the infusion of FVIII concentrate in haemophilia A patients results in lowered VWF plasma levels. However, the impact of F8-deficiency on VWF plasma levels in F8-/y mice is unresolved. In order to avoid confounding variables, we back-crossed F8-deficient mice onto a pure C57BL/6J background and analysed VWF plasma concentrations relative to C57BL/6J WT (F8+/y) littermate controls...
2017: PloS One
https://www.readbyqxmd.com/read/28836262/immune-status-of-patients-with-haemophilia-a-before-exposure-to-factor-viii-first-results-from-the-hemfil-study
#19
Letícia L Jardim, Daniel G Chaves, Amanda C O Silveira-Cassette, Ana Cristina Simões E Silva, Marcio P Santana, Monica H Cerqueira, Alessandra Prezotti, Claudia Lorenzato, Vivian Franco, Johanna G van der Bom, Suely M Rezende
Previous cross-sectional studies showed that some patients with haemophilia A (HA) without inhibitor presented a pro-inflammatory profile during factor VIII (FVIII) replacement therapy. Furthermore, an anti-inflammatory/regulatory state was described in HA patients after inhibitor development. However, no study investigated the levels of these biomarkers before exposure to exogenous FVIII. This study investigated the immunological profile of previously untreated patients (PUPs) with HA in comparison with non-haemophiliac boys...
August 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28835123/advances-in-gene-therapy-for-haemophilia
#20
Amit C Nathwani, Andrew M Davidoff, Edward G D Tuddenham
Gene therapy provides hope for a cure for patients with haemophilia by establishing continuous endogenous expression of factor VIII or factor IX following transfer of a functional gene copy to replace the haemophilic patient's own defective gene. Haemophilia may be considered a 'low hanging fruit' for gene therapy because a small increment in blood factor levels (≥2% of normal) significantly improves the bleeding tendency from severe to moderate, eliminating most spontaneous bleeds. After decades of research, the first trial to provide clear evidence of efficiency after gene transfer in patients with haemophilia B using adeno-associated viral (AAV) vectors was reported by our group in 2011...
August 23, 2017: Human Gene Therapy
keyword
keyword
4856
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"