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Authors

  1. Sherry, David D.

Abstract

The most frequent, severe clinical manifestation of hemophilia is hemarthrosis, which often develops at a young age and is initiated by repeated joint bleeds. Blood within a joint results in inflammation and hypertrophy of synovial membranes, causing increased vascularization of the joint and bone degeneration. In addition, hemarthrosis is associated with pain, impaired mobility, and reduced health-related quality of life (HRQoL). Although data on the effect of joint pain on pediatric HRQoL are currently lacking, both health-related outcome and treatment-experience studies reported the benefits of early identification, assessment, and treatment of pain in improving patient outcome and HRQoL. Ultimately, prevention of bleeding through aggressive treatment of joint bleeds and prophylaxis with factor concentrates are key to ensuring the best musculoskeletal outcome for pediatric patients with hemophilia.