A longitudinal study of orthopaedic outcomes for severe factor‐VIII‐deficient haemophiliacs

  title={A longitudinal study of orthopaedic outcomes for severe factor‐VIII‐deficient haemophiliacs},
  author={Louis M Aledort and R H Haschmeyer and Holger Pettersson},
  journal={Journal of Internal Medicine},
Abstract. Objective. Arthropathy is the major cause of morbidity in haemophilia. In an attempt to establish optimal therapeutic regimens for persons with haemophilia, we hypothesized that no direct relationship exists between increasing factor dosage and orthopaedic outcomes over time. 

Paper Mentions

Observational Clinical Trial
Hemophilia is a bleeding disorder (deficiency of a blood clotting factor/ protein) resulting in bleeding in joints and muscles. As patients continue to bleed into their joints they… Expand
ConditionsHemarthroses, Hemophilia A, Hemophilia B
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In the 1950s, Sweden initiated prophylaxis as a lifelong treatment for haemophilia and was the first country to do so, according to the World Health Organization. Expand
Effect of late prophylaxis in hemophilia on joint status: a randomized trial
SPINART was a 3‐year randomized clinical trial of late/tertiary prophylaxis vs on‐demand therapy that improved function, quality of life, activity and pain but not joint structure by MRI. Expand
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Whether age at first index joint bleed, blood group, FVIII gene abnormality variant, factor VIII trough level, first‐year bleeding rate and adherence to the prescribed prophylaxis regimen significantly predicted joint damage trajectory, and thus class membership, is assessed. Expand
Management of joint bleeding in hemophilia
The current management of hemophilic joint bleeding is reviewed and the potential impact of novel therapies are discussed. Expand
Bleed volume of experimental knee haemarthrosis correlates with the subsequent degree of haemophilic arthropathy
The individual pathological response to the same number of clinically evident joint bleeds is highly variable; thus, it remains unknown if certain joint bleeding characteristics are critical for the development of arthropathy. Expand
Outcome measures in haemophilia: more than just factor levels
P1624. 9 Smith PS, Teutsch SM, Shaffer PA, Rolka H, Evatt B. Episodic versus prophylactic infusions for haemophilia A: a cost–effectiveness analysis. J Pediatr 1996; 129:
Pattern of bleeding in a large prospective cohort of haemophilia A patients: A three‐year follow‐up of the AHEAD (Advate in HaEmophilia A outcome Database) study
Outcome data on treatment of patients with haemophilia A spanning several years of real‐world evidence collection are currently very limited.
Prospective audit of patients with haemophilia: Bleeding episodes and management
This poster focuses on the treatment of children with haemophilia with a history of atypical or recurrent bleeding and the management of these episodes through on‐demand therapy. Expand
Haemophilia: A race for cure
The mainstay of treatment continues to be replacement of the missing factor by infusion of FVIII and FIX concentrates, either on demand in response to bleeding episodes or as prophylaxis. Expand


Twenty‐five years' experience of prophylactic treatment in severe haemophilia A and B
It appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1% of normal. Expand
The natural history of factor VIII:C inhibitors in patients with hemophilia A: a national cooperative study. II. Observations on the initial development of factor VIII:C inhibitors.
Seventeen of the 31 inhibitors, including 12 of 15 with maximum values greater than 10 Bethesda U/mL, developed within 75 exposure days to factor VIII within the course of a 4-year study of acquired factor VIII inhibitors in persons with hemophilia A. Expand
Diagnostic Imaging in Hemophilia
Diagnostic imaging in hemophilia , Diagnostic imaging in hemophilia , کتابخانه دیجیتالی دانشگاه علوم پزشکی و خدمات درمانی شهید بهشتی
Twenty-five years experience of prophylactic treatment in severe haemophilia
  • A and B. journal of Internal Medicine
  • 1992
California: (Children's Hospital of Los Angeles, Los References I KernofTP. Current Factor VIII Usage Related to Self Sufficiency: A European Overview. Factor VIII: Purity and Prophylaxis
  • The United States of America: Massachusetts: (The Memorial Hospital
  • 1991
Current Factor VIII Usage Related to Self Sufficiency: A European Overview. Factor VIII: Purity and Prophylaxis
  • Royal Society of Medicine Services,
  • 1991
Musculoskeletal and other haemorrhagic complications
  • Diagnostic Imaging in Hemophilia. Berlin: Springer-Verlag
  • 1985
Investigators : England : (Trelor Haemophilia Centre, Lord Mayor Trelor College) Tony Aronstam MD : (Northern Regional Haemophilia Service, The Royal Victoria Infirmary
  • Holger Pettersson MD (University Hospital