ESPE Abstracts (2019) 92 RFC6.2

High Levels of LIGHT/TNFSF14 in Prader-Willi Syndrome

Giacomina Brunetti1, Danilo Fintini2, Antonino Crinò3, Laura Piacente4, Alessio Convertino2, Silvia Concetta Colucci1, Maria Grano5, Graziano Grugni6, Maria Felicia Faienza7


1Department of Basic Medical Sciences, Neuroscience and Sense Organs, Section of Human Anatomy and Histology, University "A. Moro" of Bari, Bari, Italy. 2Dipartimento Pediatrico Universitario Ospedaliero, BambinoGesù Children's Hospital, Tor Vergata University, Roma, Italy. 3Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Roma, Italy. 4University of Bari, A. Moro, Bari, Italy. 5Department of Emergency and Organ Transplantation, University of, Bari, Italy. 6Istituto Auxologico Italiano, IRCCS, Division of Auxology and Metabolic Diseases, Piancavallo (VB), Italy. 7Department of Biomedical Sciences and Human Oncology, University A.Moro of Bari, Bari, Italy


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.

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