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https://www.readbyqxmd.com/read/27919671/supracondylar-femoral-osteotomy-and-knee-joint-replacement-during-the-same-surgical-procedure-in-a-type-a-haemophiliac-patient-with-knee-flexion-deformity-and-ankylosis
#1
Jose Luis Osma Rueda, Alejandra Oliveros Vargas, Cristian David Sosa
BACKGROUND: Haemophilia A is the cause of diverse musculoskeletal disorders such as ankylosis, arthritis and associated angular deformity. There are few reported cases in patients with haemophilia A in which simultaneous supracondylar femoral osteotomy and knee joint replacement has been performed to treat knee angular deformity and ankylosis. Here we present the case of an 18year old male patient, with an evolution of two years, who was unable to walk due to the presence of an untreated supracondylar fracture in the left femur and ipsilateral haemophilic arthropathy which led him to develop an ankylosis in flexion close to 70°...
December 2, 2016: Knee
https://www.readbyqxmd.com/read/27919470/spontaneous-sublingual-haematoma-in-acquired-haemophilia-case-report
#2
T Spindler, N Mc Goldrick, J McMahon, R Campbell Tait
Acquired haemophilia is a rare disease in which bleeding is more severe than in hereditary haemophilia and usually occurs in the soft tissues, the gastrointestinal tract, or the mucous membranes. There have been only a few presentations of spontaneous sublingual haematoma in acquired haemophilia, but prompt management of the airway and identification of the underlying cause was crucial in all.
December 2, 2016: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/27914734/applying-a-deliberation-model-to-the-analysis-of-consultations-in-haemophilia-implications-for-doctor-patient-communication
#3
Giulia Lamiani, Sarah Bigi, Maria Elisa Mancuso, Antonio Coppola, Elena Vegni
OBJECTIVE: Literature highlights the importance of communication in order to achieve patient's adherence. However, the specific dialogical components likely to favor patient adherence are not clear. In this study, the deliberation dialogue model was applied as an ideal model of optimal deliberation to real physician-patient consultations in the field of hemophilia in order to identify misalignments with the model and possible improvements in physician-patient communication. METHODS: By applying the deliberation model, we analyzed a corpus of 30 check-up consultations in hemophilia...
November 28, 2016: Patient Education and Counseling
https://www.readbyqxmd.com/read/27904904/clinical-evaluation-of-glycopegylated-recombinant-fviii-efficacy-and-safety-in-severe-haemophilia-a
#4
Paul Giangrande, Tatiana Andreeva, Pratima Chowdary, Silke Ehrenforth, Hideji Hanabusa, Frank W G Leebeek, Steven R Lentz, Laszlo Nemes, Lone Hvitfeldt Poulsen, Elena Santagostino, Chur Woo You, Wan Hui Ong Clausen, Peter G Jönsson, Johannes Oldenburg
Turoctocog alfa pegol (N8-GP) is a novel glycoPEGylated extended half-life recombinant factor VIII (FVIII) product developed for prophylaxis and treatment of bleeds in patients with haemophilia A, to enable higher activity levels with less frequent injections compared with standard FVIII products. This phase III (NCT01480180), multinational, open-label, non-randomised trial evaluated the safety and clinical efficacy of N8-GP when administered for treatment of bleeds and for prophylaxis, in previously treated patients aged ≥12 years with severe haemophilia A...
December 1, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27898515/cost-effectiveness-analysis-of-pharmacokinetic-driven-prophylaxis-vs-standard-prophylaxis-in-patients-with-severe-haemophilia-a
#5
Sergio Iannazzo, Paolo A Cortesi, Roberto Crea, Katharina Steinitz, Lorenzo G Mantovani, Alessandro Gringeri
The objective of this study was to assess the cost-effectiveness of pharmacokinetic-driven prophylaxis in severe haemophilia A patients. A microsimulation model was developed to evaluate the cost-effectiveness of pharmacokinetic-driven prophylaxis vs. standard prophylaxis and estimate cost, annual joint bleed rate (AJBR), and incremental cost-effectiveness ratio over a 1-year time horizon for a hypothetical population of 10 000 severe haemophilia A patients. A dose of 30 IU/kg per 48 h was assumed for standard prophylaxis...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27894230/factor-viii-delivery-devices-in-haemophilia-a-barriers-and-drivers-for-treatment-adherence
#6
Isabel Fernández-Arias, Hae Kyung Kim
INTRODUCTION AND OBJECTIVE: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). METHOD: A cross-sectional research through a supervised survey and a DCS testing session. RESULTS: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17...
November 1, 2016: Farmacia Hospitalaria
https://www.readbyqxmd.com/read/27893354/summary-report-of-the-first-international-conference-on-inhibitors-in-haemophilia-a
#7
Sebastien Lacroix-Desmazes, David W Scott, Jenny Goudemand, Marijke Van Den Berg, Michael Makris, Alice S Van Velzen, Elena Santagostino, David Lillicrap, Frits R Rosendaal, Anneliese Hilger, Zuben E Sauna, Johannes Oldenburg, Lorenzo Mantovani, M Elisa Mancuso, Craig Kessler, Charles R M Hay, Paul Knoebl, Giovanni Di Minno, Keith Hoots, Amanda Bok, Mark Brooker, Erica Buoso, Pier Mannuccio Mannucci, Flora Peyvandi
No abstract text is available yet for this article.
November 25, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27893350/the-bleeding-assessment-tool-and-laboratory-data-in-the-characterisation-of-a-female-with-inherited-haemophilia-a
#8
Viviana Daidone, Eva Galletta, Antonella Bertomoro, Alessandra Casonato
No abstract text is available yet for this article.
November 15, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27891721/extended-half-life-pegylated-full-length-recombinant-factor-viii-for-prophylaxis-in-children-with-severe-haemophilia-a
#9
E S Mullins, O Stasyshyn, M T Alvarez-Román, D Osman, R Liesner, W Engl, M Sharkhawy, B E Abbuehl
INTRODUCTION: Primary factor VIII (FVIII) prophylaxis is the optimal treatment in children with severe haemophilia A. They are expected to benefit from extended half-life (T1/2 ) FVIII coverage by reduced infusion frequency while maintaining haemostatic efficacy. AIMS: To determine immunogenicity, pharmacokinetics (PK), efficacy, safety and quality of life of prophylaxis with a polyethylene glycol (peg)-ylated FVIII (BAX 855) based on full-length recombinant FVIII (ADVATE) in paediatric previously treated patients (PTPs) with severe haemophilia A...
November 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27885373/a-new-era-of-treatment-for-patients-with-haemophilia-a
#10
Robert Klamroth
Treatment and prevention of bleeding episodes in patients with severe haemophilia A require frequent intravenous injection of factor VIII. Inhibitory antibodies against factor VIII occur in approximately 30 % of these patients during the first exposure days and immune tolerance induction to eradicate the inhibitor is challenging. Prevention of bleeds in patients with haemophilia A and inhibitors is less effective and there is ongoing research for alternative treatment options. A promising approach in 2016 is the development of emicizumab (ACE910), a bi-specific IgG antibody to factor IXa and factor X, that mimics the cofactor function of factor VIII...
November 25, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27880029/hypertension-haematuria-and-renal-functioning-in-haemophilia-a-cross-sectional-study-in-europe
#11
P A Holme, C Combescure, R C Tait, E Berntorp, S Rauchensteiner, P de Moerloose
BACKGROUND AND OBJECTIVES: This cross-sectional, epidemiological study sought to assess the prevalence and extent of potential risk factors for hypertension, particularly renal function related to haematuria and their associations in people with haemophilia. METHODOLOGY: Demographic and medical data were collected at a single time-point in patients with haemophilia over 40 years of age from 16 European centres. Associations with diagnosis of hypertension were tested in univariate and multivariate analyses...
March 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27878207/plasma-derived-versus-recombinant-factor-concentrates-in-pups-a-never-ending-debate
#12
Erik Berntorp
Inhibitor development in haemophilia is a serious complication to treatment with factor concentrates. Since the advent of more pure products, especially developed using recombinant DNA technology, some studies have shown an increased incidence of inhibitors in previously untreated patients (PUPs) receiving recombinant products whereas plasma-derived concentrates sometimes have been claimed to have a protective role, probably due to the content of von Willebrand factor (VWF). In fact, experiments indicate that the VWF may block uptake of factor VIII into macrophages for further processing to the immune system...
November 23, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27868369/evaluation-of-the-utility-of-the-isth-bat-in-haemophilia-carriers-a-multinational-study
#13
P D James, J Mahlangu, C Bidlingmaier, M E Mingot-Castellano, M Chitlur, P F Fogarty, A Cuker, M E Mancuso, P A Holme, J Grabell, N Satkunam, W M Hopman, P Mathew
INTRODUCTION: There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use of bleeding assessment tools in this population are lacking. AIM: Our objective was to validate the ISTH-BAT in haemophilia carriers to describe bleeding symptoms and allow for comparisons with factor levels and other patient groups. METHODS: This was a prospective, observational, cross-sectional study performed by members of Global Emerging HEmostasis Panel (GEHEP)...
November 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27862687/anti-factor-viii-antibodies-in-brothers-with-haemophilia-a-share-similar-characteristics
#14
J Kahle, A Orlowski, D Stichel, J F Healey, E T Parker, S M Donfield, J Astermark, E Berntorp, P Lollar, D Schwabe, C Königs
INTRODUCTION: The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is currently the most serious complication for patients with haemophilia A undergoing FVIII replacement therapy. Several genetic factors have been acknowledged as risk factors for inhibitor development. AIM: To analyze the influence of genetic factors on the nature of the humoral immune response to FVIII in eight brother pairs with inhibitors. METHODS: The domain specificity of FVIII-specific IgG was analysed by antibody binding to FVIII fragments and homologue-scanning mutagenesis (HSM)...
November 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27862662/prevalence-and-severity-by-age-and-other-clinical-correlates-of-haemophilic-arthropathy-of-the-elbow-knee-and-ankle-among-taiwanese-patients-with-haemophilia
#15
C-Y Chang, T-Y Li, S-N Cheng, R-Y Pan, H-J Wang, S-Y Lin, Y-C Chen
INTRODUCTION & AIMS: Haemophilic arthropathy (HA) is a major complication in patients with haemophilia (PWH), but the study of age-specific prevalence and severity of HA is very limited in Asian countries. MATERIALS & METHODS: This study retrospectively reviewed 146 severe- and moderate-type Taiwanese PWH aged 4-73 years, with roentgenograms of elbows, knees and ankles and calculated Pettersson scores. RESULTS: The prevalence of HA, mean number of HAs per patient and mean Pettersson scores of all the joints were 42...
November 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27860135/joint-lavage-followed-by-viscosupplementation-and-triamcinolone-in-patients-with-severe-haemophilic-arthropathy-objective-functional-results
#16
M U Rezende, F R Andrusaitis, R T Silva, E Okazaki, J D A Carneiro, G C Campos, A F Pailo, R Frucchi, T Pasqualin, P R Villaça
INTRODUCTION: Viscosupplementation can improve function in haemophilia patients. Viscosupplementation results can be improved by prior joint lavage and triamcinolone administration. AIM: To objectively assess whether viscosupplementation and associated triamcinolone use in patients with severe haemophilic arthropathy following joint lavage improves force and balance and reduces bleeding events. METHODS: Fourteen patients with haemophilic knee arthritis with and without the involvement of other joints underwent joint lavage and subsequent injections of Hylan G-F20 and triamcinolone into all affected joints...
November 16, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27852679/acquired-haemophilia-a-an-unusual-postoperative-complication
#17
Sumant Arora, Gaurav Goyal, Rehan Sarmad, Kenneth J Wool
An African-American man aged 65 years with multiple malignancies in remission was admitted for small bowel obstruction. He was treated with laparotomy following failure of conservative management. Postoperatively, he developed intra-abdominal bleed, which persisted, despite surgical haematoma evacuation. Further haematological workup revealed isolated prolongation of activated partial thromboplastin time (aPTT) with reduced factor VIII (FVIII) activity and raised FVIII inhibitor titre. Assuming acquired haemophilia A (AHA), FVIII inhibitor bypassing activity and corticosteroids were started with subsequent resolution of the bleeding from the surgical site...
November 16, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27824213/combined-coagulation-factor-viii-and-factor-ix-deficiency-cdf8f9-in-a-patient-from-lithuania
#18
V Ivaškevičius, B Pezeshkpoor, A Biswas, G Goldmann, S Horneff, M Gimbutyte, L Malciute, R Jurgutis, J Oldenburg
: Haemophilia A (FVIII deficiency) and haemophilia B (FIX deficiency) are X-linked inherited bleeding disorders. It is a very rare event to identify both haemophilias in the same patient. So far, only two families with such combination are reported in the literature worldwide supported by genetic background. PATIENTS AND METHODS: Evaluation of clinical data, determination of FVIII and FIX levels and genetic analysis of F8 and F9 genes by direct sequencing. RESULTS: We report on a patient having severe haemophilia B (FIX:C <1 IU dl(-1)) and mild haemophilia A (FVIII:C 18 IU dl(-1) )...
November 8, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27824212/regulatory-t-cells-and-their-potential-for-tolerance-induction-in-haemophilia-a-patients
#19
A Schmidt, C Königs
FVIII inhibitors still are the major concern in treatment of haemophilia A patients by FVIII replacement therapy. Immune tolerance induction to reverse inhibitor formation fails in about 30% of treated patients. These patients face increased morbidity and mortality producing a need for new therapy strategies in the treatment of FVIII inhibitor-positive patients. Regulatory T cells are important modulators of the immune response and are also involved in the immune response to FVIII in haemophilia A patients...
November 8, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27824209/mutational-spectrum-and-deep-intronic-variants-in-the-factor-viii-gene-of-haemophilia-a-patients-identification-by-next-generation-sequencing
#20
J E Bach, J Oldenburg, C R Müller, S Rost
: Haemophilia A (HA) is caused by a broad spectrum of different mutation types in the factor VIII gene (F8). In our patient cohort of more than 2600 HA patients as well as in other published studies, the most frequent cause are missense mutations in different F8 exons or the recurrent intron 22 inversion. Some exons and several specific nucleotide positions represent hot spots for point mutations in the examined cohort. About 4 % of cases remain without mutation after routine HA diagnostic methods including inversion PCRs, Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA)...
November 8, 2016: Hämostaseologie
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