Comparative study of dual energy X‐ray absorptiometry and quantitative ultrasonography with the use of biochemical markers of bone turnover in boys with haemophilia

Abstract

Our aim was to evaluate bone status in boys with haemophilia using dual energy X-ray absorptiometry (DXA) and quantitative ultraSonography (QUS), and in addition, to compare these two methods with the use of biochemical markers of bone turnover. Twenty-six boys with a mean decimal age of 12.08 ± 4.44 years were included in the study which included a DXA scan at lumbar spine and radial, as well as tibial QUS. Serum levels of soluble receptor activator of nuclear factor κB ligand (sRANK-L), osteoprotegerin (OPG) and osteocalcin (OC) were measured and joint evaluation was performed using the Hemophilia Joint Health Score (HJHS). With regard to the study results, only 2 of 26 patients (7.7%) had bone mineral density (BMD) Z-scores < -2, and 4 patients (15.4%) had BMD Z-scores between -1 and -2. Only one patient had radial and other two had tibial QUS Z-scores < -2. No agreement was recorded between QUS and DXA in identifying patients at risk for osteoporosis (k = 0.275, P = 0.063). Haemophiliacs had significantly higher serum levels of sRANK-L (21.04 ± 4.78 vs. 18.58 ± 2.28 ng mL(-1), P = 0.038) and of OC (5.35 ± 2.29 vs. 3.09 ± 0.61 ng mL(-1), P = 0.002) and significantly decreased levels of OPG (15.78 ± 2.53 vs. 23.79 ± 4.39 pg mL(-1), P < 0.001) compared with controls. QUS Z-scores at tibia significantly correlated with HJH Scores (r = -0.450, P = 0.040), whereas lumbar BMD Z-scores significantly correlated with body mass index Z-scores (r = 0.500, P = 0.009). More studies are warranted to identify the most accurate densitometric method for assessing bone status in haemophiliacs.

RANK-RANKL-OPG in Hemophilic Arthropathy: From Clinical and Imaging Diagnosis to Histopathology

“…To date, few studies have addressed the role of these molecules in HAt 19,21 . To date, few studies have addressed the role of these molecules in HAt 19,21 .…”

“…The few studies that have addressed the measurement of serum levels of sRANKL and OPG in patients with HAt have shown varying results 18,19 . Several researchers have demonstrated that serum OPG concentrations increase with age in both women and men 33,34 , but in our samples there were no differences of OPG serum levels related to age (data not shown).…”

“…In RA, characterized by both inflammation and bone erosions, the interaction between the RANK-RANKL-OPG system and T cells has been demonstrated 10,11 . Therefore, high bone resorption seems not to be balanced by comparable bone formation 18,19 . A high prevalence of osteoporosis among hemophilic patients has been reported; the condition is related to the severity of arthropathy and is enhanced by human immunodeficiency virus (HIV) infection.…”

Clinical and imaging analysis of subclinical hemophilia combined with coxarthrosis: case report and literature review

“…Additionally, X-ray imaging cannot directly show the articular cartilage and the changes can be only presumed through joint space narrowing. Therefore, X-ray examination and scoring is more applicable for the hemophilic arthropathy with bone lesion (Christoforidis et al 2011). …”

Outcome measures monitoring physical function in children with haemophilia: a systematic review

“…Hilliard et al [49] 0 + + 0 Christoforidis et al [50] 0 0 0 À Feldman et al [43] + + 0 + Groen et al [31] + 0 0 0 Groen et al [51] 0 0 0 À +, ICC or k ≥ 0.70; ?, inadequate statistical method; À, no correlation or statistical difference or ICC/k < 0.70; 0, no information about test-retest reliability; N/A, not applicable.…”