View clinical trials related to Haemophilia.
Filter by:Research project for the development, construction and validation of a questionnaire of adherence to treatment in adult patients with hemophilia. It aims to build a scale according to five relevant dimensions of adherence to treatment: 1) physician-patient relationship, 2) knowledge of the disease, 3) previous bleeding problems, 4) knowledge of future consequences and difficulties, and 5) benefits of treatment.
Psychosocial factors and adherence to treatment in patients with hemophilia. A multicenter study. Multicenter cross sectional study of patients with hemophilia and their families
Occurrence of inhibitors to coagulation factor VIII is diagnosed in ~30% patients with haemophilia A. Presence of inhibitor with a titre >5 BU/ml requires the use of by-passing agents: recombinant activated Factor VIIa concentrate (rFVIIa) and/or activated prothrombin complex concentrate (APCC). Similarly, haemorrhagic complications in patients with acquired haemophilia and inhibitor titre >5 BU/ml should be treated with by-passing agents. Response to treatment with by-passing agents is patient-specific, and can vary in the same patient during subsequent bleedings. Some patients have good response to both products, however in other patients a better bleeding control is provided by one of the mentioned above agents (APCC or rFVIIa). There are clinical situations when severe bleedings requires an alternate use of both these agents. Traditional methods of laboratory tests used post-treatment in patients with haemophilia without inhibitors are useless in the presence of inhibitor. Laboratory monitoring of therapy with by-passing agents is possible with the use of global tests for the coagulation process assessment, which are as follows: thrombin generation assay (TGA) and thromboelastometry (TEM). Several studies revealed that TGA allows a monitoring of therapy with by-passing agents in patients with haemophilia A and inhibitor - the choice of the most effective treatment option - agent type and its dose, as well as laboratory assessment of treatment efficacy. Up to date, laboratory tests assessing the efficacy of by-passing agents in patients with acquired haemophilia were not conducted. In Factor VIII or IX deficiency conditions, fibrin's fibres generated by thrombin are morphologically thicker, and blood clots have increased susceptibility to fibrinolytic enzymes. Blood clot stability may be assessed with the use of thromboelastometry (TEM). We can hypothesize that simultaneous use of TGA and TEM methods may allow for an assessment of patient's individual response to therapy with by-passing agents. Clinical significance of the minimal dose of APCC and rFVIIa, needed to TGA and TEM normalization, requires further studies. Tests' purpose: Examination of the hypothesis that simultaneous use of thrombin generation assay (TGA) and thromboelastometry (TEM) may facilitate the choice of optimal therapy with by-passing agents and laboratory monitoring of efficacy of those agents in patients with acquired haemophilia or haemophilia A with inhibitor.
The primary aim of this study is to evaluate the overall quality of life in adult patients with severe haemophilia (an inherited bleeding disorder affecting mainly men) in Turkey and to assess the impact of certain disease and drug related factors (treatment modality, presence of viral disease, inhibitor, etc.) on the quality of life.
Life expectancy of hemophilia patients has improved considerably during the past decades and is approaching that of the general population. Hemophilia patients are therefore likely to be confronted with age-related disorders in addition to their primary illness and related diseases. Little is known about the occurrence of age-related co-morbidity, especially cardiovascular disease (CVD), in these patients. Low clotting factor levels are hypothesized to protect against both atherosclerosis and thrombus formation, resulting in a reduced risk of ischemic CVD. CVD mortality has been reported to be lower in haemophilia patients than in the general population, but data on non-fatal CVD are lacking, and no adjustment for CVD risk factors has been made so far. The aim of our study is to assess the occurrence of CVD and its risk factors in a large cohort of haemophilia patients. In this prospective multicenter cohort study in a group of 700-800 male patients with haemophilia A or B aged 30 years or older from The Netherlands and the UK, data on CVD history and CVD risk factors will be collected at baseline and compared with the general age-matched male population. Overall QRISK2 cardiovascular risk scores will be calculated and also compared with the general population. During a follow-up period of 5 and 10 years the occurrence of CVD events will be recorded and compared with the expected occurrence based on the QRISK2 scores and with data from the general population.