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Published on: April 11, 2018
Cystic fibrosis related bone disease in children: Can it be predicted?
Halime Nayir Buyuksahin1, Deniz Dogru1, Onur Gözmen2
1Division of Pulmonology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Bone density in children with cystic fibrosis (CF) is not predicted by common bone turnover biomarkers. Fat-free mass index (FFMI) and hand grip strength (HGS) are the best indicators of bone mineral density (BMD) in these patients.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Cystic Fibrosis Research
Background:
- Cystic fibrosis (CF)-related bone disease (CFBD) is a significant complication.
- Low bone mineral density (BMD) in childhood CF predicts future bone disease.
- Investigating bone turnover biomarkers and predictive factors for BMD in children with CF is crucial.
Purpose of the Study:
- To investigate bone turnover biomarkers (osteocalcin, RANKL, OPG) in relation to BMD in children with CF (cwCF).
- To evaluate factors affecting bone turnover, including fat-free mass (FFM), lung function (FEV1), and hand grip strength (HGS).
Main Methods:
- Compared 16 children with CF and moderate low BMD (group1) to 64 children with CF and normal BMD (group2).
- Measured serum levels of osteocalcin (OC), receptor activator of nuclear factor kappa B ligand (RANKL), and osteoprotegerin (OPG).
- Assessed body mass index (BMI), fat-free mass index (FFMI), forced expiratory volume in 1 second (FEV1), and hand grip strength (HGS).
Main Results:
- No significant differences in serum RANKL, OC, OPG, or RANKL/OPG ratio between groups.
- Significant differences observed in BMI z-score, FFMI z-score, FEV1 z-score, and HGS %pred between groups.
- Multivariate regression identified FFMI z-score and HGS %pred as the sole predictors of BMD.
Conclusions:
- Serum OC, OPG, RANKL, and RANKL/OPG ratio do not predict BMD in children with CF.
- FFMI z-score and HGS %pred are the most effective, non-invasive predictors of BMD in this population.
Background&Aims:
Cystic fibrosis (CF) -related bone disease (CFBD) is an important complication of CF, and low BMD in childhood is a precursor of CFBD. Here, we aimed to investigate bone turnover biomarkers, including osteocalcin (OC), receptor activator of nuclear factor kappa B ligand (RANKL) and osteoprotegerin (OPG) in relation to low BMD in children with CF (cwCF). We also evaluated factors which could affect bone turnover with particular emphasis on fat-free mass (FFM), forced expiratory volume in 1 s (FEV1), hand grip strength (HGS), and functional capacity and physical activity.
Methods:
Sixteen cwCF aged 8-18 years with moderate low BMD (group1) and 64 cwCF with normal BMD (group2) were enrolled. Serum RANKL, OC, and OPG were determined by immunoenzymatic assays. Multiple parameters including pancreatic status, lung functions, body mass index (BMI), FFM measured by bioelectric impedance analysis (BIA), 6-minute walk test, vitamin D, nutritional intake, HGS, functional capacity and physical activity, serum and urine biomarkers were compared between the two groups.
Results:
We found similar serum levels of RANKL (p = 0.501), OC (p = 0.445), OPG (p = 0.380), and RANKL/OPG ratio (p = 0.449) between group1 and group2 in cwCF. BMI z-score (p < 0.001), FFMI z-score (p < 0.001), FEV1 z-score (p = 0.007), and right-HGS (%pred) (p = 0.009) significantly differed between the two groups. Multivariate linear regression revealed that the only factors that predicted BMD were FFMI z-score and HGS %pred.
Conclusion:
Serum OC, OPG, RANKL and RANKL/OPG ratio did not predict BMD in cwCF. FFMI z-score and HGS %pred measured by non-invasive and practical methods were the best predictors of BMD.
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