Background: Low bone mineral density (BMD) has been found in up to 50% of adolescents and adults with Prader-Willi syndrome (PWS). High fracture risk has been described in adult PWS patients. However, the mechanism/s of low BMD in PWS have not been clarified. These patients also display high BMI-SDS that prompted us to evaluate the levels of LIGHTTNFSF14, a cytokine involved in pathological bone remodeling and obesity.
Objective and Hypotheses: To evaluate the levels of LIGHT/TNFSF14 in PWS children and to correlate them with parameters of obesity and bone quality.
Method: Nineteen PWS children were enrolled (5M/14F, 11.35 ± 6.76) and LIGHT/TNFSF14 levels were measured in the sera. Bone status, lean mass and fat mass were assessed by DXA.
Results: Significant higher LIGHT/TNFSF14 levels were found in PWS patients than controls (426.73 ± 374.63 pg/ml vs 162.26 ± 72.47 pg/m, P< 0.006). LIGHT/TNFSF14 levels significantly correlated with IGF-1 (r=-0.534 P<0.04), PTH (r=0.393 P<0.04), femoral Z-score (r=-0.437 P<0.04), lumbar-z-score (r=-0.711 P<0.01), lean mass % (r=-0.656 P<0.02), total fat (r=0.597 P<0.04), trunk fat % (r=0.638 P<0.03), left arm fat (r=0.779 P<0.002), right arm fat (r=0.697 P<0.01).
Conclusion: We demonstrated high serum levels of LIGHT/TNFSF14 in PWS children that correlated with both fat and bone mass. This cytokine could contribute to obesity and bone disease affecting PWS patients, therefore representing a good pharmacological target.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology