Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp68 | Bone development/growth and fracture repair | ECTS2013

Long bone phenotypic analyzes of a RANK transgenic mouse line

Baaroun Vanessa , Coudert Amelie , Marty Caroline , Castaneda Beatriz , Berdal Ariane , Descroix Vianney

Introduction: Bone metastasis pathophysiology is currently defined as a vicious circle. Indeed, tumor cells co-express RANK-L and its receptor, RANK, allowing their own proliferation and peritumoral bone resorption necessary to the lesion expansion. Odontogenic tumors, which occur within the jaw, seem to present the same pathophysiological characteristics. Thus, studying the impact on bone of RANK over-expression by the osteoclasts would permit a better understanding of those ...

ba0001pp400 | Osteoporosis: treatment | ECTS2013

Osteonecrosis of the jaw and non-malignant disease

Coudert Amelie , Lescaille Geraldine , Baaroun Vanessa , Azerad Jean , Cohen-Solal Martine , Berdal Ariane , Descroix Vianney

Bisphosphonates (BP) are powerful bone resorption inhibitors. They are used for the symptomatic treatment of malignant osteolytic bone disease (e.g. multiple myeloma and bone metastasis), as well as bone diseases associated with high bone resorption (e.g. postmenopausal osteoporosis, cortisone-induced osteoporosis). However, recent data showed that a rare, but serious, adverse effect of BP therapy is osteonecrosis of the jaw (BRONJ). Given the increasing number of persons rece...

ba0006p186 | (1) | ICCBH2017

Is cherubism a systemic disease? Prospective study about 9 patients

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...

ba0005p474 | Paediatric bone disease | ECTS2016

Defining a new severity classification and searching for a pronostic factor in cherubism: NFATc1 localization is the answer

Kadlub Natacha , Siessecq Quentin , Galmiche Louise , Vazquez Marie-Paule , Badoual Cecile , Berdal Ariane , Picard Arnaud , Descroix Vianney , Coudert Amelie

Cherubism is a rare genetic disease (OMIM #118400) characterized by a massive jaw bone osteolysis. This pathology appears around 2–5 years old and in the less severe cases spontaneously regresses after puberty. So far the only treatment available is surgery, often disabling and traumatic. As the cherubism pathophysiology is not yet understood, we carried out a thoroughly characterization of the cherubism granulomas from ten unrelated patients to determine the cells involv...

ba0004op5 | (1) | ICCBH2015

Osteoclast phenotype of giant multinucleated cells in cherubism may determine the disease aggressiveness

Kadlub Natacha , Sessiecq Quentin , Lehalle Daphne , Badoual Cecile , Majouffre Claire , Berdal Ariane , Vazquez Marie-Paule , Descroix Vianney , Picard Arnaud , Coudert Amelie

Cherubism is a rare genetic disorder characterized by extensive growth of a bilateral granuloma of the jaws, resulting in facial disfigurement.Histologically, the lesions consist of a fibrotic stroma with osteoclastic-like multinuclear giant cells (MGC). Cherubism is caused by gain-of-function mutations in the SH3BP2 protein. SH3BP2 is an intracellular adaptor protein positively regulating the activity of the nuclear factor of activated T-cells c1 (NFATc...