ECE2014 Oral Communications Bone, calcium & vitamin D (5 abstracts)
High spontaneous osteoclastogenesis in pediatric osteogenesis imperfecta patients receiving or not intravenous neridronate
Maria Felicia Faienza 1 , Albina Tummolo 2 , Laura Piacente 1 , Rita Fiaschetto 2 , Maria Ciccarelli 1 , Annamaria Ventura 1 , Francesco Papadia 2 , Silvia Colucci 3 , Maria Grano 3 , Luciano Cavallo 1 & Giacomina Brunetti 3
1Department of Pediatrics, Hospital Giovanni XXIII-Policlinico of Bari, University “A.Moro” Bari, Bari, Italy; 2Section of Endocrinology and Metabolism, Hospital Giovanni XXIII-Policlinico of Bari, Bari, Italy; 3Department of Basic Medical Sciences, Neuroscience and Sense Organs, Section of Human Anatomy and Histology, University ‘A. Moro’ Bari, Bari, Italy.
Background: Osteogenesis imperfecta (OI) is a heritable disease of the connective tissues caused primarily by heterogeneous mutations in the genes encoding for type I collagen. Phenotypically, it is characterized by abnormal bone mineralization, tissue fragility, and skeletal deformities.
Objective: The aim of this study was to investigate the osteoclastogenic potential of unfractionated peripheral blood mononuclear cells (PBMCs) from OI patients (mean age 10.44±3.48) who received cyclical neridronate infusions for at least 1 year, untreated OI patients and control subjects.
Methods PBMCs from six patients and six controls were cultured in presence/absence of M-CSF and RANKL. At the end of the culture period, mature multinucleated OCs were identified as TRAP+ cells. By real-time PCR we studied gene expression in freshly isolated PBMC. By flow cytometry we characterized the presence of OC precursors (CD14+/CD16+) and we studied TNF-alpha expression.
Results: Spontaneous formation of osteoclasts, without adding M-CSF and RANKL, occurred in PBMC cultures from treated and untreated OI patients. In these patients, the percentage of circulating osteoclast precursors, CD14+/CD16+ cells, increased respect to the controls (12.5% vs 0.1%, P<0.01). By RT-PCR, we found high levels of RANKL, TNF-alpha and MCSF receptor, as well as decreased OPG levels, thus leading to the increase of RANKL/OPG ratio. High TNF-α levels were also found on monocytes through flow cytometry.
Conclusion: We showed for the first time the high osteoclastogenic potential of PBMCs from young OI patients, treated and untreated with bisphosphonate, which could be due to the high percentage of circulating OC precursors, to the elevated TNF-alpha levels as well as to the increased RANKL/OPG ratio. This condition could contribute to the bone disease affecting these patients.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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