This study describes health-related quality of life (HRQoL) of persons with haemophilia A in the United States (US) and determines associations between self-reported joint pain, motion limitation and clinically evaluated joint range of motion (ROM), and between HRQoL and ROM. As part of a 2-year cohort study, we collected baseline HRQoL using the SF-12 (adults) and PedsQL (children), along with...

With the introduction of prophylaxis, restricting children with haemophilia to participate in physical activities was no longer necessary. Subsequently, many studies report on improved physical functioning in children and adolescents with haemophilia. However, little is known about psychological aspects such as perceived competence and impact of disease. Therefore, the aims of this study were t...

INTRODUCTION haemophilia is an inherited bleeding disorder caused by a deficiency in one of the blood coagulation factors. For people affected by severe haemophilia, the deficiency can cause spontaneous internal bleeding. Most young people with severe haemophilia in the UK follow a preventative treatment regimen (prophylaxis) consisting of several intravenous injections of factor concentrate ea...

Improvements in the management of haemophilia have led to a significant increase in the life expectancy of haemophilia patients, which is now close to the life expectancy in the general male population. Therefore, age-related conditions, especially cardiovascular disease (CVD), have become increasingly common in these patients. The management of CVD, especially that of coronary artery disease (...

INTRODUCTION Adherence to prophylactic treatment in haemophilia is important for patient outcome. AIM This study analysed the influence of potential impact factors on adherence assessed through the application of the German translation of the VERITAS-Pro questionnaire. METHODS All members of the German haemophilia patient organisation (DHG) who suffer from severe or moderate haemophilia and...

Haemophilia A is an inherited, sex-linked disorder in which coagulation factor VIII (FVIII) is deficient or absent [1]. The hallmark of the severe form of the disease, defined as plasma FVIII level of <1% of normal [2], is early, recurrent bleeding into soft tissues and joints [3]. Intra-articular bleeding (haemarthrosis) accounts for more than 90% of all serious bleeding events in patients wit...

prevalence of coagulopathies (n=6010) including factor viii deficient (haemophilia a) individuals (n=3205) were investigated and analysed in 24 provinces of iran. for all coagulopathies, the prevalence (per 100,000) was the highest with 24.45 in semnan (north east of iran), followed by hamadan (west) with 17.56, the lowest was observed in kordestan (west) as 0.88, followed by kohkiluyeh & boyr-...

BACKGROUND Severe haemophilia is associated with major psychological and economic burden for patients, caregivers, and the wider health care system. This burden has been quantified and documented for a number of European countries in recent years. However, few studies have taken a standardised methodology across multiple countries simultaneously, and sought to amalgamate all three levels of bur...

1Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal 2 ICVS/3B’s – PT Government Associate Laboratory, Braga/Guimarães, Portugal 3School of Psychology, University of Minho, Braga, Portugal 4Faculty of Psychology and Education Sciences, University of Porto, Porto, Portugal 5Portuguese Haemophilia Association, Lisbon, Portugal 6European Hae...