Bone density and health-related quality of life in adult patients with severe haemophilia

摘要: Severe haemophilia and reduced bone density can negatively influence perception of patient's health-related quality life (HRQoL), especially considering future aspects, the risk losing independence or pain suffering. The aim this study was to assess levels HRQoL in severe patients compare those general population as well determine whether is correlated perceived HRQoL. Patients were divided into two groups based on timing being treated with prophylaxis: Group A (started prophylaxis at age ≤ 3 years; n = 22); B (at >3 15). mineral (BMD g cm(-2)) different measured sites by dual energy X-ray absorptiometry (DXA). assessed using SF-36 questionnaire. have mean BMD T-score >-1.0 (i.e. normal score) all sites, almost similar scores domains compared reference population. -1.0 lumbar spine total body, their lower Moreover, significant correlations found between femoral neck body physical domains. With adequate long-term since early childhood, adult report a comparable Swedish Reduced group impaired health, which underscores importance onset prophylactic treatment.