Hemophilia without prophylaxis: Assessment of joint range of motion and factor activity

Research and Practice in Thrombosis and Haemostasis

J. Michael Soucie¹  Michael Wang²  David L. Cooper³  Neeraj N. Iyer³  Michael Recht⁴ 

[1] Centers for Disease Control and Prevention Atlanta Georgia USA;Hemophilia and Thrombosis Center University of Colorado School of Medicine Aurora Colorado USA;Novo Nordisk Inc.Clinical, Medical, and Regulatory Affairs Plainsboro New Jersey USA;The Hemophilia Center at Oregon Health & Science University Portland Oregon USA;

关键词: arthropathy;    clotting factor;    hemophilia;    joint disease;    range of motion;   

DOI  :  10.1002/rth2.12347

来源: DOAJ

【 摘 要 】

Abstract Background Recurrent joint bleeding in hemophilia results in arthropathy and functional impairment. The relationship of arthropathy development and factor activity (FA) has not been reported in patients with FA levels <15%‐20%. Methods During the Centers for Disease Control and Prevention Universal Data Collection, joint range‐of‐motion (ROM) measurements were taken at each comprehensive visit. Data were extracted from male patients with hemophilia (PWH) age ≥2 years with baseline factor activity levels ≤40%, excluding those prescribed prophylaxis, and used to calculate a proportion of normal ROM (PN‐ROM) measure. Data were analyzed using regression models. Results There were 6703 eligible PWH with 30 102 visits. PN‐ROM declined with increasing age, and was associated with hemophilia severity, race/ethnicity, obesity, and viral illnesses. PWH ≥30 years old with fFA ≤2% and those ≥50 years old with FA ≤5% had mean PN‐ROM values >10% less than controls; those ≥40 years old with FA <1% had values >20% less than controls. In the multivariable analysis, subjects with <1% FA had a 0.43% greater decrease (−0.49 to −0.37, 95% confidence interval) in PN‐ROM each year relative to those with 16%‐40% factor activity. A less pronounced effect was seen with 1%‐5% or 6%‐9% FA. Conclusion The effect of FA on ROM loss is far greater than that of any of the other characteristics, especially with FA <10%. This emphasizes the need to maintain a high index of suspicion for arthropathy in individuals with moderate and low‐mild hemophilia.

【 授权许可】

Unknown