Searchable abstracts of presentations at key conferences on calcified tissues

ba0002p41 | (1) | ICCBH2013

Management of a new case of neonatal hypocalciuric hypercalcemia related to mutation of the calcium-sensing receptor gene with bone abnormalities

Edouard Thomas , Mouly Celine , Mimoun Emmanuelle , Gennero Isabelle , Magdelaine Corinne , Salles Jean Pierre

Background: A 5-month-old girl was referred to our unit after a systemic screening for hip dislocation by X-rays revealed bilateral femoral bowing. She was the first child of healthy non-consanguineous parents, and her family history was unremarkable. Her parents had a normal physical examination, and normal laboratory findings. At presentation, her height was 64.0 cm (Z-score: 0.0) with a regular height velocity. Weight was 7.4 kg (Z-score: 1.0). On physical...

ba0002p125 | (1) | ICCBH2013

Growth plate modifications in lysophosphatidic acid LPA1 receptor-invalidated mice

Gennero Isabelle , Laurencin-Dalicieux Sara , Conte-Auriol Francoise , Briand-Mesange Fabienne , Chun Jerold , Salles Jean-Pierre

Objectives: Lysophosphatidic acid (LPA) is a potent lipid growth factor which possess several G protein-coupled receptors LPA1-6. We have recently demonstrated that LPA1 receptor-invalidated mice display abnormal bone development and osteoporosis, suggesting abnormal endochondral ossification. We have here further studied the growth plates of LPA1 receptor-invalidated mice.Methods: We performed a microscopic and immuno-histochemistry analysis of the femo...

ba0002p131 | (1) | ICCBH2013

Ghrelin differentiates human osteoblasts via GHS-R1a receptor

Gennero Isabelle , Barre Ronan , Conte-Auriol Francoise , Beton Nicolas , Salles Jean Pierre

Objectives: Ghrelin is a peptide hormone secreted in the stomach, which stimulates GH release and food intake. It is also known to have an effect on bone metabolism. The ghrelin specific receptor, GHS-R1a, belongs to the G protein-coupled receptors (GPCRs). Its downstream pathway in osteoblasts remains unclear. We attempted to clarify the way by which ghrelin acts on osteoblast differentiation.Methods: We studied two human osteosarcoma cell lines, MG63 a...

ba0002p122 | (1) | ICCBH2013

Fasting total ghrelin levels are increased in patients with adolescent idiopathic scoliosis

Gennero Isabelle , Conte-Auriol Francoise , Mus Marianne , Molinas-Cazals Catherine , Accadbled Franck , Tauber Maithe , De Gauzy Jerome Sales , Salles Jean Pierre

Objectives: Ghrelin is an orexigenic hormone produced by the stomach that reflects body weight changes and stimulates GH secretion. Recently, it has been shown to be associated with bone metabolism and eating behaviour. The underlying pathophysiology of adolescent idiopathic scoliosis (AIS) refers to possible abnormal bone development. AIS patients also frequently present with low BMI level.Eating behavioural disorders, endocrine disorders, abnormal grow...

ba0004p71 | (1) | ICCBH2015

Muscle and bone impairment in children with Marfan syndrome: correlation with age and FBN1 genotype

Haine Elsa , Tauber Maithe , Van Kien Philippe Khau , Auriol Francoise , Gennero Isabelle , Julia Sophie , Dulac Yves , Salles Jean-Pierre , Edouard Thomas

Background: Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutation in the gene encoding the extracellular matrix protein fibrillin-1 (FBN1), leading to transforming growth factor-beta (TGF-β) signaling dysregulation. Although decreased axial and peripheral bone mineral density (BMD) has been reported in adults with MFS, data about the evolution of bone mass during childhood and adolescence are limited.Objectives: The aim of th...