Exploration of the pathogenesis of haemophilic joint arthropathy: understanding implications for optimal clinical management

摘要: Summary Haemophilia is an inherited disorder of clotting factor deficiencies resulting in musculoskeletal bleeding, including haemarthroses, leading to orthopaedic complications. The pathogenesis haemophilic joint arthropathy continues be explored and there evidence suggest that iron, cytokines, neo angiogenesis can initiate synovial early cartilage damage molecular changes the perpetuation a chronic inflammatory state. This has long term consequences for bone health pain quality life issues individual with haemophilia. Haemarthroses prevented by administration concentrates (prophylaxis). However, high costs need venous access devices younger children continue complicate recommendations universal prophylaxis. In patients who fail or refuse prophylaxis, procedures, such as synovectomy arthroplasty, provide relief from repeated haemarthroses. optimal timing these, however, not well defined. Prevention needs focus on prevention haemarthroses through identifying disease use cost effective imaging modalities validation serological markers arthropathy. Screening effects management improve are, likewise, important issues.