INTRODUCTION Management of haemophilia and inherited bleeding disorders is a major challenge especially in developing countries, because of a shortage or absence of products, the cost and the infrastructural health problems. Development of local expertise which results in an improved outlook and reduction in mortality and morbidity in these countries can be helpful for advocators in other devel...

Inhibitor development continues to be a major problem in the treatment of haemophilia. Immune tolerance induction (ITI) continues to be the most effective approach to managing this complication. This study reviews the practice and outcome of ITI at a single centre over a 17-year period. All 31 inhibitor patients have haemophilia A. Two patients with haemophilia A underwent two trials of ITI and...

The primary goal of prophylaxis in patients with severe haemophilia is to convert the phenotype from severe to moderate and to prevent the development of chronic arthropathy. Prior studies have demonstrated that prophylaxis decreases episodes of joint bleeds and chronic arthropathy. Effectiveness depends on prescription of prophylaxis and adherence to the prescribed regimen. The aim of this stu...

In comparison with other biotechnology substitutions, the adoption of recombinant Factor VIII (rFVIII) has been relatively slow. We sent a postal questionnaire to all Dutch haemophilia patients and haemophilia-treating physicians, to determine which factors predict whether a patient uses plasma-derived FVIII (pdFVIII) or rFVIII and to investigate patients' and doctors' opinions on both products...

Musculoskeletal dysfunction is a common feature of haemophilia and along with other manifestations of this condition, there is a general perception that health-related quality of life (QoL) will be affected. Previous research using standardized questionnaires has demonstrated that QoL is lower in haemophilia groups compared with normal populations. However, disability studies and interviews wit...

BACKGROUND Although it is widely appreciated that vigorous physical activity can increase the risk of bleeding episodes in children with haemophilia, the magnitude of the increase in risk is not known. Accurate risk estimates could inform decisions made by children with haemophilia and their parents about participation in physical activity and aid the development of optimal prophylactic schedul...

: Risk for thrombotic events with factor IX replacement therapy in patients with haemophilia B remains a concern for patients, those who treat them, and regulatory agencies, based on experience with early use of prothrombin complex concentrates. The current post hoc analysis assessed the incidence of thrombotic events and changes in prothrombin fragment 1 + 2, thrombin-antithrombin complex, and...

BACKGROUND Children with haemophilia have lower levels of fitness and strength than their healthy peers. We present the protocol of a study designed to determine whether an exercise intervention improves quality of life, aerobic fitness and strength in children with haemophilia. METHODS/DESIGN The study will be a randomised, assessor-blinded, controlled trial of exercise treatment. Seventy ch...

Although there is a worldwide interest in the assessment of health-related quality-of-life (HRQoL) in haemophilia patients, no non-disease specific instruments (for adults) are readily available. In this paper, a haemophilia-specific quality-of-life assessment measure for adults (the Hemofilia-QoL questionnaire) has been developed and tested for psychometric properties in 121 adults with haemop...

ACW LEE MBBS, FHKAM Abstract A survey was performed in late-2003 by questionnaire to all paediatric and adolescent departments under the Hospital Authority of Hong Kong to study the epidemiology and clinical status of local haemophilia patients under 19 years of age. A total of 90 patients were recruited, corresponding to a local prevalence of 6.4 per 100,000. Among these 90 patients, 83.3% wer...