Searchable abstracts of presentations at key conferences on calcified tissues

ba0005oc3.2 | Clinical trials, FGF-23 and focal osteoporosis | ECTS2016

Effects of Denosumab (Dmab) on bone matrix mineralization: results from the phase 3 FREEDOM trial

Dempster David W , Brown Jacques P , Yue Susan , Farlay Delphine , Rizzo Sebastien , Song Jenny , Wang Andrea , Wagman Rachel B , Boivin Georges

Low fracture (FX) incidence has been demonstrated in women with postmenopausal osteoporosis (PMO) treated with DMAb for up to 10 years in the FREEDOM extension [Bone ASBMR 2015]. Bone biopsy-based assessment of DMAb’s effects at the tissue level has demonstrated a low remodelling rate consistent with DMAb’s mechanism of action (Reid JBMR 2010; Brown JBMR 2014). From FREEDOM, we report the effects of DMAb on bone matrix mineralization in women who un...

ba0006oc1 | (1) | ICCBH2017

NBAS variants causing a novel form of inherited bone fragility

Balasubramanian Meena , Hurst Jane , DeVile Catherine , Bishop Nick , Arundel Paul , Offiah Amaka , Pollitt Rebecca , Hughes David , Longman Dasa , Caceres Javier , Skerry Tim

Background: Osteogenesis imperfecta (OI), the commonest inherited bone fragility disorder, affects 1/15,000 live births resulting in frequent fractures and reduced mobility, with significant impact on quality of life. Early diagnosis is important, as therapeutic advances can lead to improved clinical outcomes.Methodology and results: Trio whole exome sequencing in patients with OI identified, in two patients, compound heterozygous mutations in NBAS</...

ba0006oc14 | (1) | ICCBH2017

Body composition and associated biomarkers as determinants of bone mineral density in children 6–8 years of age – The Physical Activity and Nutrition in Children (PANIC) study

Soininen Sonja , Sidoroff Virpi , Lindi Virpi , Mahonen Anitta , Kroger Liisa , Kroger Heikki , Jaaskelainen Jarmo , Atalay Mustafa , Laaksonen David , Laitinen Tomi , Lakka Timo A.

Background and objectives: Lean mass (LM) has been positively associated with bone mineral density (BMD), but the impact of increased adiposity on bone especially in children is controversial. Several biomarkers, secreted by adipose tissue, skeletal muscle, or bone, may have important roles in the bone health. Our aim was to study the association of body composition, adipokines, myokines, inflammation-related cytokines, growth factors, and serum 25-hydroxyvitamin D (S-25(OH)D)...

ba0006oc22 | (1) | ICCBH2017

Type I collagen C-propeptide cleavage deficiency increases bone mineralization and alters bone cell differentiation

Barnes Aileen , Perosky Joseph , Blouin Stephane , Rajpar M. Helen , Khoury Basma , Weis MaryAnn , Klaushofer Klaus , Roschger Paul , Eyre David , Fratzl-Zelman Nadja , Kozloff Kenneth , Marini Joan

High Bone Mass (HBM) osteogenesis imperfecta (OI) is caused by dominant mutations in the C-propeptide cleavage site of COL1A1 or COL1A2, characterized by bone hypermineralization. To elucidate the role of C-propeptide processing in bone mineralization and development, we generated heterozygous HBM mice with both residues (Ala-Asp) of the COL1A1 cleavage site substituted (Thr-Asn) to prevent processing by BMP1. Two, 6- and 12-month WT and HBM bones were examin...

ba0006p118 | (1) | ICCBH2017

Genome-wide association study identifies five novel genetic determinants of dental maturation

Grgic Olja , Vucic Strahinja , Medina-Gomez Carolina , Trajanoska Katerina , Dhamo Brunilda , Jaddoe Vincent , Ongkosuwito Edwin , Jarvelin Marjo-Riitta , Timpson Nicholas , Evans David , Wolvius Eppo , Rivadeneira Fernando

Objectives: Advanced or delayed physiological age may influence significantly health and disease processes. Physiological age can be estimated using several parameters including dental age (DA). Previous meta-analyses studying “Number of Teeth at 15 Months” (NT15M) and “Age at First Teeth Eruption” (AFTE) have identified 15 loci. We performed a genome-wide association study (GWAS) meta-analysis to identi...

ba0007oc8 | (1) | ICCBH2019

Bisphosphonate improves hip range of motion and pain but not femoral head sphericity: A multicentre, randomized clinical trial of children with Perthes disease

Jamil Kamal , Zacharin Margaret , Foster Bruce , Donald Geoffrey , Hassall Timothy , Siafarikas Aris , Johnson Michael , Tham Elanie , Whitehead Colin , Gebski Val , Barnes Liz , Cowell Chris , Little David , Munns Craig

Introduction: Perthes disease (PD), idiopathic femoral head avascular necrosis, often results in deformity. The underlying cause is unclear and long-term function is directly related to the roundness of femoral head. Current treatment include mechanical treatments and various surgical procedures, which are therapeutic but can’t prevent collapse. A multicentre, prospective, randomised controlled trial of 12 months zoledronic acid (ZA) in children with PD was conducted. We ...

ba0002p22 | (1) | ICCBH2013

Association of volumetric bone mineral density, bone morphometry and trabecular bone micro-architecture with leptin and soluble leptin receptor in adolescent idiopathic scoliosis

Tam Elisa M S , Yu Fiona W P , Hung Vivian W Y , Liu Zhen , Lam Tsz-Ping , Liu King Lok , Ng Bobby K W , Lee Simon K M , Qiu Yong , Cheng Jack C Y

Objectives: Low bone mass in adolescent idiopathic scoliosis (AIS) has been well reported, however the etiologies of the disease and this abnormal phenotype were still unknown. Leptin have profound effects on bone metabolism and skeletal growth, and was speculated to play a role in the etiopathogenesis of AIS. The objective of this study was to investigate the bone quality in AIS and its association with leptin and soluble leptin receptor (sOB-R).Methods...

ba0004p60 | (1) | ICCBH2015

Comparing two scanning protocols for high-resolution peripheral quantitative computed tomography for bone quality assessment in young subjects

Cheuk Ka Yee , Zhang Jiajun , Tsang Echo , Yu Fiona , Hung Vivian , Tam Elisa M S , Lam Tsz Ping , Lee Simon K M , Ng Bobby K W , Cheng Jack C Y

Objectives: High resolution peripheral quantitative computed tomography (HR-pQCT) was used to evaluate bone quality and volumetric bone mineral density (vBMD). However, manufacturers only provide a standard protocol for adult but not for growing subjects. The aim of this study was to investigate the difference on measurement between two different HR-pQCT scanning protocols in adolescence with unfused growth plate.Methods: 27 boys and 26 girls aged 13&#15...

ba0001pp205 | Cell biology: osteoblasts and bone formation | ECTS2013

Thrombin receptor deficiency leads to osteopetrosis by decreasing the RANKL/OPG ratio

van der Eerden BCJ , Tudpor K , Jongwattapapisan P , Woudenberg-Vrenken TE , Bindels RJM , Hoenderop JGJ , van Leeuwen JPTM

Communication between osteoblasts and osteoclasts is crucial for bone remodeling. Thrombin and its thrombin receptor (TR; PAR-1) are expressed in osteoclasts and osteoblasts, respectively. To date, the physiological roles of thrombin and TR in bone metabolism have not been elucidated. Therefore, we fully characterized the bone phenotype of mice lacking the thrombin receptor.We performed bone microarchitectural analyses of the femurs of 10–12 week ol...