Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p72 | (1) | ICCBH2019

Burosumab experience in UK X-linked hypophosphataemia children under five years old

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone and rickets. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We report relevant real-world biochemical data on children under five years ...

ba0007p73 | (1) | ICCBH2019

Burosumab initiation in a UK X-linked hypophosphataemia cohort: real-world use resonates with research evidence

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone resulting in rickets, skeletal abnormalities, physical impairment, weakness, and pain. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We...

ba0005oc4.1 | Catabolism and metabolism | ECTS2016

A small molecule inhibitor of TRAF6 dependent signaling reduces osteoclastogenesis and prevents ovariectomy induced bone loss

Marino Silvia , Bishop Ryan , Bendle Gavin , Nicolaes Gerry , Lutgens Esther , Idris Aymen

Tumour necrosis factor receptor-associated factor 6 (TRAF6) plays a key role in osteoclastogenesis through the regulation of RANK/CD40 TRAF6-mediated signaling. Mice deficient in TRAF6 exhibit high bone mass and were protected against inflammation-induced bone loss. Here we describe the effects of a small-molecule 6877002 that has been shown to prevent the binding of TRAF6 to its domain on RANK/CD40 receptor on osteoclast formation in vitro and in vivo and on...

ba0003pp314 | Osteoporosis: treatment | ECTS2014

The effect of risedronate on hypogonadal osteoporotic HIV males treated with highly active anti-retroviral therapy: a pilot study

Pepe Jessica , Isidori Andrea M , Falciano Mario , Iaiani Giancarlo , Salotti Alessandra , Diacinti Daniele , Del Fiacco Romano , Sbardella Emilia , Cipriani Cristiana , Piemonte Sara , Raimo Orlando , D'angelo Antonella , Romagnoli Elisabetta , Lenzi Andrea , Minisola Salvatore

Objective: We evaluated the effect of risedronate on BMD and bone turnover markers in HIV infected osteoporotic males, according to their gonadal status.Methods: 41 HIV patients treated with highly active anti-retroviral therapy (HAART) were followed for 24 months and divided into two groups: patients with osteoporosis or osteopenia with fractures (Group A, n=20) and those without (Group B, n=21). Group A and B were fur...

ba0001pp31 | Bone biomechanics and quality | ECTS2013

A GWAS in an extreme high bone mass population shows excess signal from genes associated with BMD in the normal population

Gregson Celia L , Leo Leo Paul J , Clark Graeme R , Smith George Davey , Brown Matthew A , Tobias Jon H , Duncan Duncan Emma L

Extreme high bone mass (HBM) may be monogenic (e.g. due to mutations in SOST or LRP5) or polygenic, due to variants in the same genes determining bone mineral density (BMD) as found in the general population. We aimed to determine the genetic cause underlying HBM in an extreme HBM population.258 unexplained HBM cases (defined as L1 Z-score ≥+3.2 plus total hip Z-score ≥+1.2, or total hip Z-score ≥+3.2 and L1 <...

ba0001pp43 | Bone biomechanics and quality | ECTS2013

Protective effect of polyphenols from Aronia melanocarpa berries against cadmium-induced weakening of the femur biomechanical properties in rats

Brzoska Malgorzata M , Roszczenko Alicja , Rogalska Joanna

Bone damage is one of the main unfavourable health effects of chronic exposure to cadmium (Cd). This heavy metal stimulates osteoclastic bone resorption and inhibits osteoblastic bone formation resulting in decreased bone mineralization and as a result weakening of the bone biomechanical properties. Recently, using a rat model of chronic human exposure to cadmium, we have revealed that even low exposure to this metal may increase bone vulnerability to fracture. Taking into acc...

ba0001pp227 | Cell biology: osteoclasts and bone resorption | ECTS2013

RANKL immobilized on β-TCP induces and maintains osteoclast formation

Choy John , Hofstetter Wilhelm , Klenke Frank M

β-tricalcium phosphate (β-TCP) biomaterials have been approved for the repair of osseous defects. However, in large defects, the substitution of biomaterial by authentic bone is inadequate to provide sufficient long-term mechanical stability. We aimed to develop composites of β-TCP ceramics and receptor activator of nuclear factor κ-B ligand (RANKL) to enhance the formation of osteoclasts thereby stimulating material resorption. RANKL was immobilized on &#9...

ba0001pp273 | Genetics | ECTS2013

Functional analysis of the two Runx3 promoters in osseous and non-osseous cells: implications for tissue/differentiation specific transcription of distinct isoforms

Conceicao Natercia , Simoes Brigite , Cancela M Leonor

The Runt-domain transcription factors Runx2 and Runx3 are known to drive chondrocyte maturation from prehypertrophic to the terminal stage. The RUNX family proteins form dimers with CBFb, and bind to consensus sequences of 5′-PuACCPuCa-3′ upstream of target genes to activate or repress transcription.To address the role of Runx3 transcription factor in zebrafish, we have isolated the different splice variants encoding distinct runx3 p...

ba0001pp296 | Muscle, physical activity and bone | ECTS2013

Body composition relationship in Korean old people

Choi Woong H , Hong Sang M

Background: Recently, the prevalence of osteoporosis and sarcopenia in the elderly has dramatically increased. However the relationship between theses disease is not clear.Object: We aimed to determine the independent relations of muscle mass to osteoporosis (femur neck) in relation to body weight, fat mass, and other confounders.Design: We analyzed body composition and BMD data of 570 males and 734 females who are older than 65 ye...