ba0006p186 | (1) | ICCBH2017
Joly Aline
, Maruani Gerard
, Daire Valerie Cormier
, Fauroux Brigitte
, Berdal Ariane
, Picard Arnaud
, Coudert Amelie
Introduction: Cherubism is a rare pediatric disease with a maxillofacial localization caused by mutations of the SH3BP2 gene. Pathogenesis is well described in the Sh3bp2 KI mouse model that presents a systemic inflammatory and bone phenotypes maintained by TNFα and due to the presence of hypersensitive myeloid precursors. In human, the disease is usually described as a maxillofacial exclusive disease. The aim of our study was to explore the systemic phe...