Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp282 | Genetics | ECTS2013

Phenotypic dissection of bone mineral density facilitates the identification of skeletal site specificity on the genetic regulation of bone

Kemp John P , Medina-Gomez Carolina , Estrada Karol , Heppe Denise , Zillikens Carola , Timpson Nicholas , Pourcain Beate , Ring Susan , Hofman Albert , Jaddoe Vincent V W , Smith George Davey , Uitterlinden Andre G , Tobias Jonathan H , Rivadeneira Fernando , Evans David M

Heritability of bone mineral density (BMD) varies at skeletal sites, possibly reflecting different relative contributions of environmental and genetic influences. To quantify shared genetic influences across different sites, we estimated the genetic correlation of BMD at the upper limb (UL), lower limb (LL), and skull (S) obtained from whole body DXA scans, using bivariate genome-wide complex trait analysis (GCTA). The study (n=9395) combined data from the Avon Longit...

ba0005oc1.4 | Clinical trials and osteoporosis treatment | ECTS2016

Relationship between total hip (TH) BMD T-score and incidence of nonvertebral fracture (NVFX) with up to 10 years of Denosumab (Dmab) treatment

Ferrari S , Adami S , Brown J P , Cosman F , Czerwiński E , de Gregorio L H , Malouf J , Reginster J-Y , Daizadeh N S , Wang A , Wagman R B , Lewiecki E M

The relationship between BMD T-score and FX risk has not been established in patients receiving osteoporosis therapy. In the FREEDOM Extension study, continuous DMAb therapy for up to 10 years increased BMD levels with no therapeutic plateau at lumbar spine or TH [Bone et al, ASBMR 2015]. Such improvements would only be meaningful if associated with FX reductions. We investigated the relationship between TH BMD T-score and NVFX in women who received DMAb during FREEDOM and tho...

ba0002oc26 | Chronic diseases | ICCBH2013

Vertebral fractures in the 3-year period following steroid initiation among children with chronic illnesses

Miettunen P M , Taljaard M , Alos N , Atkinson S , Cabral D , Clarson C , Couch R , Cummings E A , Feber J , Grant R M , Lentle B , Matzinger M , Nadel H , Rodd C , Shenouda N , Stein R , Stephure D , Taback S , Rauch F , Siminoski K , Ward L M , the Canadian STOPP Consortium

Objectives: To describe the incidence of vertebral fractures in steroid-treated children.Methods: Fractures were assessed prospectively each year for 3 years according to the Genant semi-quantitative method. Proportions of children with incident fractures were determined annually over the study period. To examine associations with baseline clinical factors, the 3-year total number of incident fractures was analyzed using multivariable Poisson regression....

ba0005ht5 | (1) | ECTS2016

Superior Gains in Bone Mineral Density (BMD) and Estimated Strength at the Hip for Romosozumab Compared With Teriparatide (TPTD) in Women With Postmenopausal Osteoporosis Transitioning From Bisphosphonate Therapy: Results of the Phase 3 Open-label STRUCTURE Study

Langdahl B , Libanati C , Crittenden D B , Bolognese M A , Brown J P , Daizadeh N S , Dokoupilova E , Engelke K , Finkelstein J S , Genant H K , Goemaere S , Hyldstrup L , Jodar-Gimeno E , Keaveny T M , Kendler D , Lakatos P , Maddox J , Malouf J , Massari F E , Molina J F

STRUCTURE was a phase 3, open-label study evaluating the effect of romosozumab or TPTD for 12 months in women with postmenopausal osteoporosis transitioning from bisphosphonate therapy (NCT01796301). This study enrolled women with postmenopausal osteoporosis who had taken an oral bisphosphonate for ≥3 years prior to screening and alendronate in the year prior to screening; had a BMD T-score ≤−2.5 at the total hip (TH), lumbar spine (LS), or femoral neck (FN);...

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...

ba0002is5biog | Rare diseases | ICCBH2013

Acrodysostosis

Linglart Agnes

Biographical DetailsDr A Linglart is a Paediatric Endocrinologist working at the Hôpital St Vincent de Paul in Parism, France. She has a special interest in rare diseases....

ba0001pp241 | Cell biology: osteocytes | ECTS2013

Inhibition of osteocyte-induced osteoclast precursor proliferation and migration by mechanical strain

Ko Seong-Hee , Lee Heesu

The osteocyte most likely plays a role in bone remodeling by instructing osteoclasts to remove bone at specific sites. This entire process includes recruitment, proliferation and differentiation of osteoclast precursors. And osteocytes are responsible for detecting and responding to mechanical strain and may send signal to other cells. Therefore, to determine the role for osteocytes and mechanical strain in bone remodeling, we examined the effect of steady or pulsatile shear s...

ba0001pp86 | Bone development/growth and fracture repair | ECTS2013

The effect of incubation time of preformed injectable hydrogels on bone formation when used as carrier of rhBMP-2

Piskounova Sonya , Hulsart-Billstrom Gry , Gedda Lars , Bergman Kristoffer , Hilborn Jons , Larsson Sune , Bowden Tim

Introduction: Hydrogels has demonstrated efficacy as carriers for growth factors. Our aim was to investigate the effect of curing-time of modified hyaluronan on bone formation.Methods: Hydrogels with rhBMP-2 were cross-linked for 14 and 3 days, 5 h or 1 min before injection. Preformed gels were injected s.c. in 5 rats, the rats were killed after 5 weeks. Explanted samples were radiographed and scanned by pQCT.Results: Bone formatio...

ba0004op7 | (1) | ICCBH2015

Lessons from homocystinuria: Cystathionine beta-synthase as a novel marker for osteogenic differentiation of human mesenchymal stem cells

Gambari Laura , Manferdini Cristina , Gabusi Elena , Paolella Francesca , Lisignoli Gina , Mariani Erminia , Grassi Francesco

Objective: Classical homocystinuria due to cystathionine beta-synthase (CBS) deficiency, is a rare autosomal recessively inherited disease characterized by the multiple involvement on different organs. While the most striking cause of morbidity and mortality is thromboembolism, patients develop a marked osteoporosis at early age along with many other skeletal abnormalities. As CBS normally converts homocysteine to cystathionine, the result of CBS deficiency is an accumulation ...