Bone turnover markers, osteoprotegerin and RANKL cytokines in children with cystic fibrosis

Purpose: Some scientific studies show decreased bone mineral density and increased fracture frequency in adult patients with cystic fibrosis (CF). The mechanism for early bone loss in CF patients are multifactorial: chronic pulmonary inflammation, malnutrition, reduced physical activity, delayed pubertal maturation. The aim of this study was to assess bone metabolism markers with special attention paid to osteoprotegerin (OPG) and receptor activator of nuclear factor kappa B ligand (RANKL) balance in CF children. Material and Methods: The study included 35 children with diagnosed CF and 35 healthy controls aged 5-9 years (median 7.0 years). Serum levels of fat soluble vitamins were measured by chemiluminescence (vitamin D) and HPLC (vitamins A, E) methods. Concentrations of bone metabolism markers were determined by immunoenzymatic assay. Results: Mean levels of fat soluble vitamins (A, D, E) were lower in patients with CF compared to controls. In CF children we observed a significant (p<0.01) decrease in concentration of bone formation marker (osteocalcin) and similar bone resorption markers (CTX, TRACP5b) in comparison with healthy children. The serum level of OPG was significantly lower (p<0.05) and RANKL nearly 2-fold higher in patients with CF than in the healthy ones. The ratio of OPG to RANKL was about 2-fold lower in children with CF compared to healthy peers (p<0.01). Conclusion: In CF children, an imbalance between bone formation and resorption processes occurs. An increase serum RANKL concentration coexisting with lower levels of OPG may be associated with intensification of bone resorption.