Musculoskeletal disorders in the haemophilias.
The management of the haemophilias has been improved by the advent of potent consistent clotting factor replacement therapy. The previously lethal major complications such as intracerebral haemorrhage are now rare, and the infective complications of treatment, most notably hepatitis and AIDS, are now potentially preventable with the new synthetic products. There is also the prospect of 'cure' by gene insertion therapy. Advanced arthropathy has been minimized but not prevented by early effective treatment of haemarthroses, and there is a diminishing legacy of severely affected patients many of whom may require joint replacement surgery. The present group of such patients has a high prevalence of HIV-1 infection and an increased risk of joint sepsis. The available avenues of treatment for the subacute stage of the arthropathy have not been particularly effective, emphasizing the need to prevent recurrent bleeding. The development of a multidisciplinary team-management approach in centres of expertise has been a significant factor in the improved longevity, life satisfaction and preserved mobility now available to most haemophiliacs.
York
J R
JR
D001777:Blood Coagulation; D006405:Hematology / Q000639:trends; D006467:Hemophilia A / Q000150:complications* / Q000235:genetics / Q000628:therapy; D006801:Humans; D007592:Joint Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D009140:Musculoskeletal Diseases / Q000209:etiology*