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https://www.readbyqxmd.com/read/28220685/incidence-of-low-titre-factor-viii-inhibitors-in-patients-with-haemophilia-a-meta-analysis-of-observational-studies
#1
A Messori, F Peyvandi, D Mengato, P M Mannucci
INTRODUCTION: A few studies have been focused on low-titre inhibitors in patients with haemophilia A. Although several putative factors have been implicated in the development of these inhibitors, solid data are still lacking. AIM: The aim of this study was to perform a proportion meta-analysis on the incidence of low-titre inhibitors in haemophilia A. METHODS: We surveyed the PubMed database to identify studies on de novo development of low-titre inhibitors in haemophilia A patients...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220631/treatment-of-bleeding-episodes-with-recombinant-factor-viii-fc-fusion-protein-in-a-long-study-subjects-with-severe-haemophilia-a
#2
A D Shapiro, J N Mahlangu, D Perry, J Pasi, D V Quon, P Chowdary, E Tsao, S Li, A Innes, G F Pierce, G A Allen
INTRODUCTION: The Phase 3 A-LONG study demonstrated the safety and efficacy of rFVIIIFc for the control and prevention of bleeding episodes in severe haemophilia A. AIM: To describe the treatment of bleeding episodes with rFVIIIFc in the A-LONG study. METHODS: A-LONG subjects (<1 IU dL(-1) endogenous FVIII) were treated with individualized prophylaxis (Arm 1), weekly prophylaxis (Arm 2) or episodic treatment (Arm 3). Information recorded for each bleeding episode included type, location and dose to treat the episode...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220573/a-comparison-of-two-types-of-ankle-supports-in-men-with-haemophilia-and-unilateral-ankle-pain-from-arthropathy
#3
D Oleson, L Fox, T Nguyen, P Sochacki, M McCarthy, E Adams, M Recht
INTRODUCTION: Adults with haemophilia frequently have a painful and disordered gait due to ankle arthropathy. AIM: The aim of this study was to determine if pain and gait parameters were affected by the use of different types of ankle bracing. METHODS: We investigated the gait patterns of 17 men with severe haemophilia without bracing, using a fracture boot (FB) and a carbon fibre floor reaction ankle foot orthosis (CF-AFO). Pain relief was determined in each condition...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220555/population-pharmacokinetic-characterization-of-bay-81-8973-a-full-length-recombinant-factor-viii-lessons-learned-importance-of-including-samples-with-factor-viii-levels-below-the-quantitation-limit
#4
D Garmann, S McLeay, A Shah, P Vis, M Maas Enriquez, B A Ploeger
INTRODUCTION: The pharmacokinetics (PK), safety and efficacy of BAY 81-8973, a full-length, unmodified, recombinant human factor VIII (FVIII), were evaluated in the LEOPOLD trials. AIM: The aim of this study was to develop a population PK model based on pooled data from the LEOPOLD trials and to investigate the importance of including samples with FVIII levels below the limit of quantitation (BLQ) to estimate half-life. METHODS: The analysis included 1535 PK observations (measured by the chromogenic assay) from 183 male patients with haemophilia A aged 1-61 years from the 3 LEOPOLD trials...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205396/can-the-diagnostic-reliability-of-the-thrombin-generation-test-as-a-global-haemostasis-assay-be-improved-the-impact-of-calcium-chloride-concentration
#5
L A Parunov, S S Surov, Y Liang, T K Lee, M V Ovanesov
BACKGROUND: Thrombin generation test (TGT) is a global haemostasis assay with a potential to predict bleeding tendencies and treatment effects in patients with haemophilia. Despite 15 years of clinical research, the diagnostic value of TGT remains controversial, possibly due to suboptimal sensitivity to coagulation deficiencies, robustness and reproducibility. OBJECTIVE: The goal of this study was to explore the effect of calcium chloride (CaCl2 ) concentration on the TGT's response to intrinsic coagulation factors (F) VIII, IX and XIa...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205367/multiplex-ligation-dependent-probe-amplification-as-first-mutation-screening-for-large-deletions-and-duplications-in-haemophilia
#6
D Belvini, R Salviato, P Radossi, G Tagariello
INTRODUCTION: Molecular characterization has shown a wide mutational spectrum underlying haemophilia A (HA) and haemophilia B (HB). Different molecular assays have allowed laboratories to perform genetic testing for F8 and F9 mutations. AIM: Recently, multiplex ligation-dependent probe amplification (MLPA), a simple technique for relative quantitation of targeted genomic regions, has been introduced in HA and HB for detection of large deletions and duplications...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205285/natural-history-and-clinical-characteristics-of-inhibitors-in-previously-treated-haemophilia-a-patients-a-case-series
#7
A Iorio, A M Barbara, M Makris, K Fischer, G Castaman, C Catarino, E Gilman, K Kavakli, T Lambert, R Lassila, T Lissitchkov, E Mauser-Bunschoten, M E Mingot-Castellano, N Ozdemir, I Pabinger, R Parra, J Pasi, K Peerlinck, A Rauch, V Roussel-Robert, M Serban, A Tagliaferri, J Windyga, E Zanon
BACKGROUND: Development of inhibitors is the most serious complication in haemophilia A treatment. The assessment of risk for inhibitor formation in new or modified factor concentrates is traditionally performed in previously treated patients (PTPs). However, evidence on risk factors for and natural history of inhibitors has been generated mostly in previously untreated patients (PUPs). The purpose of this study was to examine cases of de novo inhibitors in PTPs reported in the scientific literature and to the EUropean HAemophilia Safety Surveillance (EUHASS) programme, and explore determinants and course of inhibitor development...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205277/preliminary-evaluation-of-altered-brain-microstructure-in-the-emotion-cognition-region-in-children-with-haemophilia-a-a-diffusional-kurtosis-imaging-study
#8
D Hu, H Kang, Y Lv, N Zhang, L Tang, J Zhang, K Shi, R Wu, Y Peng
AIM: Using diffusional kurtosis imaging (DKI) to assess the impact of emotional disorders on microstructural changes of the brain in children with haemophilia A. MATERIALS AND METHODS: Diffusional kurtosis imaging was acquired from haemophilia A (n = 22) and controls (n = 22) using a 3T scanner. A regression analysis of frontal, cingulate, hippocampus, insula and amygdala regions of interest (ROIs) was conducted. Clinical data and results of psychological tests were collected...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28198996/treatment-burden-haemostatic-strategies-and-real-world-inhibitor-screening-practice-in-non-severe-haemophilia-a
#9
Paul Batty, Steve K Austin, Kate Khair, Carolyn M Millar, Ben Palmer, Savita Rangarajan, Jan-Phillip Stümpel, Murugaiyan Thanigaikumar, Thynn T Yee, Daniel P Hart
Inhibitor formation in non-severe haemophilia A is a life-long risk and associated with morbidity and mortality. There is a paucity of data to understand real-world inhibitor screening practice. We evaluated the treatment burden, haemostatic strategies, F8 genotyping and inhibitor screening practices in non-severe haemophilia A in seven London haemophilia centres. In the 2-year study period, 44% (377/853) patients received at least one haemostatic treatment. Seventy-nine percent of those treated (296/377) received factor VIII (FVIII) concentrate...
March 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28198071/measuring-fviii-activity-of-glycopegylated-recombinant-factor-viii-n8-gp-with-commercially-available-one-stage-clotting-and-chromogenic-assay-kits-a-two-centre-study
#10
A Hillarp, A Bowyer, M Ezban, P Persson, S Kitchen
INTRODUCTION: Factor VIII activity (FVIII:C) assays of samples containing glycoPEGylated recombinant FVIII such as turoctocog alfa pegol (N8-GP) can be associated with differences in FVIII recovery in vitro between various one-stage activated partial thromboplastin time (APTT)-based clotting assays and some chromogenic assays. Careful validation and qualification of specific assays and conditions is therefore necessary for the assessment of FVIII:C in samples containing modified FVIII molecules...
February 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28193140/short-term-clinico-radiological-outcome-of-chronic-knee-synovitis-among-haemophilia-a-patients-post-phosphorus-32-radiosynoviorthesis
#11
Prasanna Ty, Jagadish Menon, Nataraj Ar, Dhanapathi Halanaik, Harshith Kramadhari
PURPOSE: Various modes of therapy have been directed at breaking the vicious cycle at early stage of synovitis in haemophilia patients. This study was planned to assess the short-term clinico-radiological outcome of chronic knee synovitis among haemophilia A patients post phosphorus-32 (P-32) radiosynoviorthesis. METHODS: P-32 samarium radiocolloid was injected into the knee and patients were followed up at 1 and 3 months, respectively. Clinical outcomes was assessed using Tegner Lysholm scores (TLSs), Modified Knee Society Clinical Rating System (MKSS) score and circumference of the knee joint...
January 2017: Journal of Orthopaedic Surgery
https://www.readbyqxmd.com/read/28187737/evaluation-of-a-web-based-registry-of-inherited-bleeding-disorders-a-descriptive-study-of-the-brazilian-experience-with-hemovidaweb-coagulopatias
#12
Suely Meireles Rezende, Silvia Helena Lacerda Rodrigues, Kelly Neves Pinheiro Brito, Diego Lima Quintino da Silva, Marcos Lázaro Santo, Bárbara de Jesus Simões, Guilherme Genovez, Helder Teixeira Melo, João Paulo Baccara Araújo, Danila Augusta Accioly Varella Barca
BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28182257/inhibitor-screening-in-non-severe-haemophilia-patients-a-major-challenge
#13
EDITORIAL
Anne-Mette Hvas, Lone H Poulsen
No abstract text is available yet for this article.
March 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28181369/cost-effectiveness-analysis-of-late-prophylaxis-vs-on-demand-treatment-for-severe-haemophilia-a-in-italy
#14
A Coppola, A D'Ausilio, A Aiello, S Amoresano, M Toumi, P Mathew, A Tagliaferri
INTRODUCTION: Long-term regular administrations of factor VIII (FVIII) concentrate (prophylaxis) initiated at an early age prevents bleeding in patients with severe haemophilia A (HA). The 5-year prospective Italian POTTER study provided evidence of benefits in adolescents and adults of late prophylaxis (LP) vs. on-demand therapy (OD) in reducing bleeding episodes and joint morbidity and improving quality of life; however, costs were increased. AIM: The aim of this study was to determine the cost-effectiveness of LP vs...
February 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28174142/polyarteritis-nodosa-in-a-patient-with-haemophilia-a
#15
Asuka Tsuji, Yukiko Kiniwa, Fuminao Kamijo, Tomomi Miyake, Atsuko Ohashi, Noriko Senoo, Kazuhisa Urushihata, Ryuhei Okuyama
No abstract text is available yet for this article.
February 3, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28159192/recombinant-factor-ix-fc-fusion-protein-in-children-with-haemophilia-b-kids-b-long-results-from-a-multicentre-non-randomised-phase-3-study
#16
Kathelijn Fischer, Roshni Kulkarni, Beatrice Nolan, Johnny Mahlangu, Savita Rangarajan, Giulia Gambino, Lei Diao, Alejandra Ramirez-Santiago, Glenn F Pierce, Geoffrey Allen
BACKGROUND: Kids B-LONG was a multicentre, open-label, phase 3 study assessing the safety, efficacy, and pharmacokinetics of recombinant factor IX Fc fusion protein (rFIXFc) in previously treated paediatric patients younger than 12 years with severe haemophilia B. METHODS: The study enrolled 30 previously treated boys younger than 12 years with haemophilia B (≤2 IU/dL [≤2%] endogenous coagulation factor IX [FIX] activity). All patients were initially given rFIXFc prophylaxis (50-60 IU/kg) once per week with adjustments to dose (≤100 IU/kg per infusion) or dosing frequency (up to two times per week) as needed...
February 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28155828/elbow-arthroplasty-in-patients-with-bleeding-disorders-preliminary-report-of-a-single-centre-experience
#17
Andrzej Leń, Jerzy Mirosław Jaworski, Joanna Zdziarska, Bartłomiej Skotnicki, Bogusław Frańczuk
Spontaneous or traumatic hemorrhages into joints that occur in the course of congenital bleeding disorders due to deficiencies of plasma clotting factors lead to early and extensive damage to joints. Arthropathy in those patients most commonly affects the knee, elbow and ankle. Replacement surgery of the damaged joint is the only effective way to treat such advanced changes. Although arthritis of the elbow is quite common, surgery is rarely undertaken; hence relevant reports in the literature are limited to individual or small groups of cases...
March 23, 2016: Ortopedia, Traumatologia, Rehabilitacja
https://www.readbyqxmd.com/read/28155262/comparative-burden-of-arthropathy-in-mild-haemophilia-a-register-based-study-in-sweden
#18
M Osooli, S Lövdahl, K Steen Carlsson, K Knobe, F Baghaei, M Holmström, J Astermark, E Berntorp
INTRODUCTION: Mild haemophilia is a congenital bleeding disorder affecting males. The burden of arthropathy in mild haemophilia has not been comprehensively described. AIM: The aim of this study was to compare the incidence, age at diagnosis and surgery for arthropathy and related hospitalizations between people with mild haemophilia and the general population in Sweden. METHODS: This was a register-based cohort study. Eligible participants were those with mild haemophilia born between 1941 and 2008 and a randomly selected, birthdate and sex-matched comparison group from the general population...
February 2, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28145076/low-agreement-between-fresh-and-frozen-thawed-platelet-rich-plasma-in-the-calibrated-automated-thrombogram-assay
#19
M Ljungkvist, S Lövdahl, E Zetterberg, E Berntorp
INTRODUCTION: Thrombin generation tests (TGTs) are considered to give more detailed information of the overall coagulation capability of a patient than clotting-based routine assays. The TGT thrombin generation assay-calibrated automated thrombogram (TGA-CAT) uses both platelet-poor plasma (PPP) and platelet-rich plasma (PRP). Assessing PRP gives more physiological test conditions and is of great interest considering the important role platelets play in haemostasis. However, PRP needs to be assessed close after blood draw/preparation as freezing fragments the platelets...
January 31, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28124511/ledipasvir-sofosbuvir-and-sofosbuvir-plus-ribavirin-in-patients-with-chronic-hepatitis-c-and-bleeding-disorders
#20
C E Walsh, K Workowski, N A Terrault, P E Sax, A Cohen, C L Bowlus, A Y Kim, R H Hyland, B Han, J Wang, L M Stamm, D M Brainard, J G McHutchison, A von Drygalski, F Rhame, M W Fried, P Kouides, G Balba, K R Reddy
INTRODUCTION: Chronic hepatitis C virus (HCV) infection is prevalent among patients with inherited bleeding disorders and is a leading cause of mortality in those with haemophilia. AIM: We evaluated the efficacy and safety of ledipasvir-sofosbuvir and sofosbuvir plus ribavirin in patients with chronic HCV genotype 1-4 infection and an inherited bleeding disorder. METHODS: Ledipasvir-sofosbuvir was administered for 12 weeks to patients with genotype 1 or 4 infection and for 12 or 24 weeks to treatment-experienced cirrhotic patients with genotype 1 infection...
January 25, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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