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https://www.readbyqxmd.com/read/29343899/flap-cover-in-a-patient-with-severe-haemophilia-type-a
#1
Narender Manickavachakan, Sunderraj Ellur, Vijay Thomas Mattyoo Joseph, Jonathan Victor, Cecil R Ross
Haemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is < 1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover...
May 2017: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/29341406/biosimilars-and-haemophilia
#2
EDITORIAL
A E Thomas
No abstract text is available yet for this article.
January 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29341405/haemophilia-of-the-third-age
#3
EDITORIAL
C Harrison, G Saccullo, M Makris
No abstract text is available yet for this article.
January 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29340855/the-relationship-between-target-joints-and-direct-resource-use-in-severe-haemophilia
#4
Jamie O'Hara, Shaun Walsh, Charlotte Camp, Giuseppe Mazza, Liz Carroll, Christina Hoxer, Lars Wilkinson
OBJECTIVES: Target joints are a common complication of severe haemophilia. While factor replacement therapy constitutes the majority of costs in haemophilia, the relationship between target joints and non drug-related direct costs (NDDCs) has not been studied. METHODS: Data on haemophilia patients without inhibitors was drawn from the 'Cost of Haemophilia across Europe - a Socioeconomic Survey' (CHESS) study, a cost assessment in severe haemophilia A and B across five European countries (France, Germany, Italy, Spain, and the United Kingdom) in which 139 haemophilia specialists provided demographic and clinical information for 1285 adult patients...
January 16, 2018: Health Economics Review
https://www.readbyqxmd.com/read/29337442/-angiology-and-haemostasis-highlights-on-some-new-data-in-2017
#5
Pierre Fontana, Helia Robert-Ebadi, Françoise Boehlen, Marc Righini
New data support the fact that prophylactic anticoagulation with direct oral anticoagulants is effective and safe for the long-term prevention of venous thromboembolism. This type of treatment is dedicated for a subgroup of patients only, which is described in this article. With regard to major bleedings occurring with these anticoagulants, new data have been added to those already available for idarucizumab, a specific antagonist of dabigatran. Finally, we summarize the data regarding a breakthrough molecule, emicizumab, which could potentially open a new era in the management of people with haemophilia A...
January 10, 2018: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29328905/diagnosis-and-care-of-patients-with-mild-haemophilia-practical-recommendations-for-clinical-management
#6
REVIEW
Gary Benson, Günter Auerswald, Gerry Dolan, Anne Duffy, Cedric Hermans, Rolf Ljung, Massimo Morfini, Silva Zupančić Šalek
Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it may not present for many years after birth, and that awareness, even within families, may be low. Methodological problems exist in diagnosis, such as inconsistencies in results obtained from different assays used to measure factor levels in mild haemophilia. Advances in genetic testing provide insight into diagnosis as well as the likelihood of inhibitor development, which is not uncommon in patients with mild or moderate haemophilia and can increase morbidity...
November 14, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29320534/the-endothelial-specific-isoform-of-type-xviii-collagen-correlates-to-annual-bleeding-rate-in-haemophilia-patients
#7
Nadja Gad Kjeld, Baolai Hua, Morten Asser Karsdal, Shu Sun, Tina Manon-Jensen
INTRODUCTION: The medical need in the haemophilic (HF) field to reduce bleeding incidents requires measurement of the annual bleeding rate (ABR) in haemophiliacs. Vascular rupture is associated with damage to the vascular endothelium causing exposure of the basement membrane. Endothelial cells and matrix impairment may be associated with joint bleeds and later development of HF arthropathy. Imbalanced extracellular matrix turnover is a central pathological feature in many diseases consequent to epithelial or endothelial cell damage...
2018: PloS One
https://www.readbyqxmd.com/read/29317438/replacing-the-haemophilia-gene
#8
(no author information available yet)
No abstract text is available yet for this article.
January 9, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29314552/australian-multicentre-study-of-current-real-world-prophylaxis-practice-in-severe-and-moderate-haemophilia-a-and-b
#9
J A Mason, S Parikh, H Tran, J Rowell, S McRae
INTRODUCTION: With the emergence of novel treatment products for haemophilia and an increasing focus on the benefits of pharmacokinetic driven individualized prophylaxis, robust national data with regard to current patterns of factor consumption and adherence are required. AIM: To characterize current Australian practice with regard to use of prophylactic clotting factor infusions in patients with moderate or severe haemophilia A (HA) and haemophilia B (HB). METHODS: This was a retrospective, non-interventional study utilizing Australian Bleeding Disorder Registry (ABDR) data collected over a 12 month period...
January 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29314439/low-incidence-of-factor-viii-inhibitors-in-previously-untreated-patients-with-severe-haemophilia-a-treated-with-octanate%C3%A2-final-report-from-a-prospective-study
#10
A Klukowska, V Komrska, V Vdovin, A Pavlova, M Jansen, S Lowndes, L Belyanskaya, O Walter, P Laguna
INTRODUCTION: Octanate® is a human, plasma-derived, von Willebrand factor-stabilized coagulation factor VIII (FVIII) concentrate with demonstrated haemostatic efficacy in previously treated patients with haemophilia A. AIM: This prospective, open-label study aimed to assess the immunogenicity of octanate® in previously untreated patients (PUPs). METHODS: The study monitored development of FVIII inhibitors in 51 PUPs. Tolerability, viral safety, FVIII recovery and efficacy of octanate® for the prevention and treatment of bleeds and in surgical procedures were also assessed...
January 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29282815/prevalence-of-and-risk-factors-for-cerebral-microbleeds-among-adult-patients-with-haemophilia-a-or-b
#11
H Husseinzadeh, T Chiasakul, P A Gimotty, B Pukenas, R Wolf, M Kelty, E Chiang, P F Fogarty, A Cuker
INTRODUCTION: Cerebral microbleeds (CMBs) represent clinically silent haemorrhagic events. Cerebral microbleeds (CMBs) portend negative neurovascular and cognitive outcomes in the general population and are associated with cognitive impairment in persons with haemophilia (PWH). Prevalence, patterns, and risk factors for CMBs in PWH have not been directly compared to persons without coagulopathy. AIM: To examine prevalence, patterns, and risk factors for CMBs in PWH vs normal controls...
December 28, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29243367/vaccinations-are-not-associated-with-inhibitor-development-in-boys-with-severe-haemophilia-a
#12
H Platokouki, K Fischer, S C Gouw, A Rafowicz, M Carcao, G Kenet, R Liesner, K Kurnik, G E Rivard, H M van den Berg
BACKGROUND: Inhibitor development in previously untreated patients (PUPs) with severe haemophilia A is a multifactorial event. It is unknown whether paediatric vaccinations given in close proximity to factor VIII (FVIII) are associated with inhibitor development. OBJECTIVE: To assess whether paediatric vaccinations in close proximity to FVIII within the first 75 exposure days (EDs) are associated with inhibitor development in PUPs with severe haemophilia A. METHODS: We included 375 PUPs with severe haemophilia A (<0...
December 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29233844/gene-therapy-boosts-factor-ix-activity-and-reduces-bleeding-events-in-patients-with-haemophilia-b-study-finds
#13
Susan Mayor
No abstract text is available yet for this article.
December 12, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29232254/orthotropic-live-transplantation-for-cirrhosis-from-hepatitis-c-virus-leads-to-correction-of-factor-ix-deficiency-allowing-for-ankle-arthroplasty-without-factor-replacement-in-a-patient-with-moderate-haemophilia-b
#14
Kumiko Ono, Jun Hirose, Song H Chang, Minoru Kubota, Junya Kinkawa, Megumi Noguchi, Hideyuki Takedani
: Liver transplantation is one of the treatments for haemophilic patients having severe liver cirrhosis who are infected with the hepatitis C virus. Patients with haemophilia can develop arthroplasty requiring surgical intervention, and the surgical outcomes of patients undergoing such procedures after liver transplant has not been reported. Treatment for arthropathy is important for improving the quality of life for patients who survive after liver transplantation. We report the first case of ankle arthroscopic arthrodesis in a patient with haemophilia B after undergoing living donor liver transplantation...
December 11, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29218759/impact-of-pain-and-functional-impairment-in-us-adults-with-haemophilia-patient-reported-outcomes-and-musculoskeletal-evaluation-in-the-pain-functional-impairment-and-quality-of-life-p-fiq-study
#15
C L Kempton, M Recht, A Neff, M Wang, T W Buckner, A Soni, D Quon, M Witkop, L Boggio, R Z Gut, D L Cooper
INTRODUCTION: Standardized and disease-specific patient-reported outcome (PRO) instruments assessing pain, functional impairment and health-related quality of life (HRQoL) in people with haemophilia (PWH) have been used in studies, but infrequently in comprehensive care settings for individual assessment or treatment planning. AIM: To assess the impact of pain and functional impairment on HRQoL in PWH. METHODS: P-FiQ enrolled 381 adult PWH with a history of joint pain/bleeding and included 5 PROs and a clinical joint evaluation (Hemophilia Joint Health Score v2...
December 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29212866/acquired-haemophilia-a-complicating-alemtuzumab-therapy-for-multiple-sclerosis
#16
Georgia McCaughan, Jennifer Massey, Ian Sutton, Jennifer Curnow
Alemtuzumab is a highly efficacious therapy used in the treatment of multiple sclerosis (MS), but uncoupling of T and B cell repopulation during immune reconstitution associates with an increasing range of secondary B cell-mediated autoimmune complications. A 34-year-old woman developed Graves' disease 11 months following an initial course of alemtuzumab treatment for MS. Nine months following the second treatment with alemtuzumab, the patient presented with spontaneous intramuscular and subcutaneous haemorrhage due to development of an inhibitory autoantibody to coagulation factor VIII...
December 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29212115/risk-factors-for-the-progression-from-low-to-high-titres-in-260-children-with-severe-haemophilia-a-and-newly-developed-inhibitors
#17
Maria Elisa Mancuso, Kathelijn Fischer, Elena Santagostino, Johannes Oldenburg, Helen Platokouki, Cristoph Königs, Carmen Escuriola-Ettingshausen, George E Rivard, Ana Rosa Cid, Manuel Carcao, Rolf Ljung, Pia Petrini, Carmen Altisent, Gili Kenet, Raina Liesner, Karin Kurnik, Günther Auerswald, Hérvè Chambost, Anne Mäkipernaa, Angelo Claudio Molinari, Mike Williams, H Marijke van den Berg
No abstract text is available yet for this article.
December 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29212114/use-of-bypassing-agents-and-risk-of-thromboembolic-events-in-patients-with-haemophilia-and-inhibitors
#18
Katsiaryna Bykov, Rhonda L Bohn, Bruce M Ewenstein, John D Seeger, Jerry Avorn, Brian T Bateman
No abstract text is available yet for this article.
December 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29212111/pathophysiological-mechanisms-of-endogenous-fviii-release-following-strenuous-exercise-in-non-severe-haemophilia-a-review
#19
C L Venema, R E G Schutgens, K Fischer
No abstract text is available yet for this article.
December 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29208927/establishment-of-a-strain-of-haemophilia-a-pigs-by-xenografting-of-foetal-testicular-tissue-from-neonatally-moribund-cloned-pigs
#20
Hiroyuki Kaneko, Kazuhiro Kikuchi, Michiko Nakai, Daiichiro Fuchimoto, Shunichi Suzuki, Shoichiro Sembon, Junko Noguchi, Akira Onishi
Grafting of testicular tissue into immunodeficient mice makes it possible to obtain functional sperm from immature donor animals that cannot be used for reproduction. We have developed a porcine model of human haemophilia A (haemophilia-A pigs) by nuclear transfer cloning from foetal fibroblasts after disruption of the X-linked coagulation factor VIII (F8) gene. Despite having a recessive condition, female F8+/- cloned pigs died of severe bleeding at an early age, as was the case for male F8-/Y cloned pigs, thus making it impossible to obtain progeny...
December 5, 2017: Scientific Reports
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