Bone Status of Children With Hemophilia A Assessed With Quantitative Ultrasound Sonography (QUS) and Dual Energy X-ray Absorptiometry (DXA)

Abstract

Recent studies report reduced bone mineral density (BMD) even among young adults and children with hemophilia. Our aim was to assess bone status in children and adolescents with hemophilia with 2 methods: Quantitative UltraSonography (QUS) and Dual energy x-ray Absorptiometry (DXA), and consequently to investigate the degree of correlation between them. Twenty-seven patients (17 with severe hemophilia; residual factor activity <1% and 10 with moderate hemophilia) participated in the study. Mean age was 12.28±4.48 y (range: 4.94 to 18.00 y). All patients were evaluated with QUS at radius and at tibia and had DXA scan at lumbar spine. Anthropometric parameters were measured using standard techniques and joint evaluation was carried out using the Hemophilia Joint Health Score (HJHS). Only 2 out of 27 patients (7.5%) had BMD Z-scores <-2, whereas another 4 patients (15%) had BMD Z-scores between -1 and -2. QUS values in both radius and tibia were generally within the normal limits as only 1 patient had radius and another 1 had tibia QUS Z-score <-2. HJH scores were significantly although negatively correlated to Z-scores of tibia QUS (r=-0.455, P=0.034). No correlations were observed between lumbar BMD and radius or tibia QUS and no agreement was recorded between QUS and DXA in identifying patients at risk for osteoporosis (k=0.262). In conclusion, our study showed that only a small number of children and young adults with hemophilia have impaired bone properties as assessed both by DXA and QUS; no correlation was observed between these 2 methods.

Bone disease in patients with haemophilia A and B – where are we now?

“…Hence, the exact prevalence of low BMD in patients with haemophilia seems to be lower than previously reported. The exact prevalence in children remains unknown, probably lower than in adults (7.5% in one study) .…”

Hypercalciuria in children with haemophilia suggests primary skeletal pathology

“…Furthermore, neither the age of prophylaxis onset nor the number of joint bleeds was reported. Another recent study (Christoforidis et al , 2010) used both quantitative ultrasound sonography (QUS) and DXA to assess bone status in 27 children or young adults (mean age 12·3 years) with moderate or severe haemophilia A, most of whom were receiving either primary or secondary prophylaxis. Their bone properties were within normal limits.…”

Bone health in persons with haemophilia: a review

“…However, low bone mass (osteopenia and osteoporosis combined) was found in 22–84% of all these patients. In general, the prevalence of osteoporosis has been found to be low in paediatric population . Gerstner et al .…”