Evaluation of bone mineral density in children with hemophilia: Mansoura University children hospital (MUCH) experience, Mansoura, Egypt

摘要

Background: Patients with hemophilia may be at risk for developing reduced bone mineral density (BMD) for a number of reasons such as recurrent hemoarthrosis and immobilization. Aim of the work: To assess the BMD in children with hemophilia and to correlate BMD with findings regarding the joint disease (hemophilic arthropathy). Patients and methods: Thirty hemophilic patients aged 4.97 ± 3.64 years and 30 control healthy individuals (without joint disease) aged 5.09 ± 3.64 years were selected from the hematology unit and outpatient clinic of MUCH respectively. Anthropometric measurements were carried out in all cases. Z-score was used for weight, height, and body mass index (BMI). Joint evaluation for hemophilic patients and controls was done using Colorado PE-0.5: Half point instrument before using dual energy X-ray absorptiometry (DEXA). DEXA scanning was performed in all hemophilic patients and controls focusing on L2-L4 vertebrae. Results: There was no significant difference between hemophilic patients and controls as regard anthropometric measurements and their Z-score. There was a significant difference between hemophilic patients and controls as regard BMD and BMD Z-score (mean ± SD) (BMD: 0.48 ± 0.13 gm/m2 for hemophilic patients vs. 0.55 ± 0.14 gm/m2 for control, p = 0.05, BMD Z-score: −0.68 ± 0.44 for hemophilic patients vs. 0.19 ± 0.14 for controls p = 0.003). There was a significant difference between severe hemophilic patients (factor level assay less than 1%) and controls as regard BMD and BMD Z-score (BMD: 0.41 ± 0.15 gm/m2 for hemophilic patients vs. 0.55 ± 0.14 gm/m2 for controls, p = 0.01, BND Z-score: −1.49 ± 0.12 for hemophilic patients vs. 0.19 ± 0.14 for controls p = 0.001). Also, in hemophilic patients, there was an inverse significant correlation between total joint evaluation scores and BMD Z-score (r = −0.365, p = 0.04). Conclusions: Children with hemophilia may have reduced BMD compared with age- and gender-matched controls. This reduction in BMD was independent of differences in age and body size. Children with more established hemophilic arthropathy exhibited the lowest BMD and BMD Z-score. Recommendations: (1) Early detection of osteopenic hemophilic children using DEXA scanning, (2) bisphosphonates plus calcium for hemophilic children with reduced BMD, (3) evaluation of the effect of on demand vs. prophylaxis replacement therapy in hemophilic patients on BMD and hemophilic arthropathy.