Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p64 | Bone development/growth and fracture repair | ECTS2016

A novel model for examining chondral bone regenerative potential in adult zebrafish

Govezensky Yael , David Dalia , Ben-Yosef Dafna , Shochat Chen , Karasik David

Purpose: The aim of this research was to develop an in vivo platform for conveniently examining the potential of various factors to augment chondral bone regeneration. For this purpose, we have established a de novo partial tail amputation model in adult zebrafish, which includes the resection of cartilage-template based bones from the endoskeletal caudal complex. Endoskeletal amputations were rarely studied, as opposed to those of the distal caudal fin rays, a well-e...

ba0007p99 | (1) | ICCBH2019

Pre and post-natal achondroplasia, retrospective series of 64 consecutives cases with analyze of the diagnostic methods and timing issues

Baujat Genevieve , Borghese Roxana , Sonigo Pascale , Bacrot Severine , Bengoa Joana , Michot Caroline , Millischer Anne-Elodie , Rondeau Sophie , Childs Beatrice , Attie-Bittach Tania , Bessieres Bettina , Salomon Laurent , Ville Yves , Bonnefont Jean-Paul , Steffann Julie , Cormier-Daire Valerie

The last years, diagnosis of achondroplasia benefited of the recent advances in prenatal imaging (including 3T-CD scan), and in invasive and non-invasive molecular screening.Objectives: To analyse stage/age, diagnosis procedures and outcome on a series of 64 consecutive cases of achondroplasia, in the French Centre of Reference for skeletal dysplasia, between 2008 and 2016.Methods: Confirmed achondroplasia were included in this sin...

ba0002is9 | (1) (1) | ICCBH2013

DXA and vertebral fracture assessment

Adams Judith

Vertebral fractures (VF) in adults are the most common osteoporotic fracture, are powerful predictors of future fracture risk (hip X2; spine X5) and their prevalence increases as bone mineral density (BMD) declines. The most common imaging method for diagnosis is spinal radiography, but they can be identified fortuitously also on other imaging techniques performed for various clinical indications.1 Midline reformations of multi-detector CT (MDCT) scans of thorax and...

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...

ba0001oc3.5 | Osteoporosis pathophysiology and genetics | ECTS2013

Genome-wide association identifies a new susceptibility locus at 4q35 associated with clinical vertebral fractures in post-menopausal women: the GEFOS-GENOMOS consortium

Alonso N , Estrada K , Herrera L , Kabir D , Olmos J M , Sanudo C , Riancho J A , Oei L , Medina-Gomez M C , Stenkjaer L , Bjerre L , Langdahl B , Brown M A , Duncan E L , Sims M , Kaptoge S , Reeve J , Lewis J , Prince R , Reppe S , Olstad O K , Gautvik K M , Garcia-Giralt N , Nogues X , Mencej-Bedrac S , Marc J , del Pino J , Gonzalez-Sarmiento R , Wolstein O , Eisman J , Feenstra B , Melbye M , Albagha O M E , WTCCC , Davies G , Starr J , Deary I , Quintela I , Fernandez C , Carracedo A , Lucas G , Elosua R , Uitterlinden A G , Rivadeneira F , Ralston S H

Vertebral fractures (VF) defined by morphometric analysis of spine radiographs are the most common complication of osteoporosis. Those that come to medical attention, with symptoms such as back pain and kyphosis are termed clinical vertebral fractures (CVF) and account for significant morbidity and mortality. Although much progress was made in identifying loci for bone mineral density, the genetic determinants of CVF remain unclear. Here we present the initial results from a g...

ba0001pp61 | Bone development/growth and fracture repair | ECTS2013

Vascularisation and progenitor cells of primary and secondary ossification centres in the human growth plate

Walzer Sonja M , Cetin Erdal , Grubl-Barabas Ruth , Sulzbacher Irene , Ruger Beate , Girsch Werner , Windhager Reinhard , Fischer Michael B

The switch from a cartilage template to bone during endochondralossification of the growth plate requires dynamic and close interaction between the cartilage and the developing vascular structures. Vascular invasion of hypertrophic cartilage, with blood vessels coming from the bone collar, serves to bring in osteoblast- andendothelial precursor cells along with chondroclasts and their precursors into future ossification centres of the growth plate.Potent...

ba0001pp160 | Cancer and bone: basic, translational and clinical | ECTS2013

Glucose ceramide synthase inhibitors prevent osteoclast activation and limit myeloma-induced osteolytic lesions

Ersek Adel , Xu Ke , Karadimitris Anastasios , Horwood Nicole J

Glycosphingolipids (GSL) are essential structural components of mammalian cell membranes and lipid rafts that exert pleiotropic effects on cell survival, proliferation, and differentiation. Cancer associated GSL have been shown to promote tumor growth, angiogenesis, and metastasis; however their role in osteoclast (OC) activation and the development of osteolytic bone diseases such as multiple myeloma are not known. We investigated the hypothesis that GSL contribute to OC acti...

ba0003pp187 | Genetics | ECTS2014

A novel mutation in IFITM5, encoding BRIL, impairs osteoblast production of PEDF and causes atypical type VI osteogenesis imperfecta

Reich Adi , Farber Charles R , Barnes Aileen M , Becerra Patricia , Rauch Frank , Cabral Wayne A , Bae Alison , Glorieux Francis H , Clemens Thomas L , Marini Joan C

Osteogenesis imperfecta (OI) type V is caused by a unique dominant mutation (c.−14C>T) in IFITM5, which encodes BRIL, a transmembrane ifitm-like protein most strongly expressed in osteoblasts, while type VI OI is caused by recessive null mutations in SERPINF1, encoding pigment epithelium-derived factor (PEDF). We identified a 25-year-old woman with severe OI, whose dermal fibroblasts and cultured osteoblasts displayed minimal secretion of PEDF, but ...

ba0003pp267 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Fracture risk and the osteoporosis treatment care gap in patients with type 1 diabetes

Khan Tayyab , Spaic Tamara , Fraser Lisa-Ann

Individuals with type 1 diabetes have over a sixfold increased risk of sustaining a hip fracture compared to the general population. Despite this, bone fragility is not recognized as a classic diabetes-related complication and many diabetes guidelines make no mention of fracture prevention or bone health.We studied bone health in a population of patients with known type 1 diabetes being followed by endocrinologists at an academic centre. Patients filled ...

ba0003pp342 | Osteoporosis: treatment | ECTS2014

Assessment treatment with denosumab in clinical practice

Torrea Maria , Carretero Lorena , Filgueira Jose Santiago

Introduction: Denosumab monoclonal antibody approved for Osteoporosis’s treatment in Europe union and U.S.A. Dose of 60 mg every 6 months reduces the risk of vertebral, non vertebral and hip fractures. What is more increases BMDMaterial and methods: Descriptive observational study with densitometric characterisques and risk factors of 64 patients in Osteoporosis unit of HGUG Marañón from November 2011 to December 2013. Analyzing occurrence...