Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp145 | Cancer and bone: basic, translational and clinical | ECTS2013

Synergistic anti-tumour effects on human breast cancer cells by mevalonate pathway inhibitors atorvastatin and zoledronic acid

Gobel Andy , Thiele Stefanie , Rauner Martina , Lorenz C Hofbauer , Tilman D Rachner

Introduction: Bone metastases represent a frequent complication of breast cancer and are characterized by increased tumour-driven activation of osteoclasts and subsequent bone loss. Aminobisphosphonates inhibit osteoclast function and are established therapies of skeletal metastases. Similar to statins, they block the mevalonate pathway and are thought to have direct anti-tumour effects. Here, we report on the anti-tumour potential of a sequential inhibition of the mevalonate ...

ba0006lb3 | (1) | ICCBH2017

Mediating effect of muscle on the relationship of physical activity trajectories and bone outcomes: The Iowa Bone Development Study

Zymbal Vera , Baptista Fatima , Letuchy Elena M. , Janz Kathleen F.

Objectives: This study analysed prospective associations between two distinct developmental trajectories of objectively-measured physical activity and late adolescent bone parameters (age 17 yr) by exploring the mediating effects of lean soft tissue (LST), a surrogate of muscle mass.Methods: In approximately 349 participants (191 girls) of the Iowa Bone Development Study, physical activity was measured by accelerometry starting at age 5 and continuing at...

ba0006p203 | (1) | ICCBH2017

Neurofibromatosis type 1 (NF1) associated congenital pseudarthrosis of the Tibia and Fibula misdiagnosed as non-accidental injury (NAI)

Mughal M Zulf , Ali Farhan , Santos Rui , Vassallo Grace , West Siobhan , Howard Elizabeth , Eelloo Judith , Hupton Eileen , Rowles Elizabeth , Huson Susan M

Background: Congenital tibial pseudarthrosis (CTP) presents with anterolateral bowing of the lower leg in infancy, which often progresses to fracture and non-union (pseudarthrosis). CTP occurs in 2–3% of children with NF1. The distal end of the fibula and other long bones can also be affected.Objective: We describe three children in whom NF1 related congenital tibial or fibular pseudarthrosis was initially misdiagnosed as NAI....

ba0002p126 | (1) | ICCBH2013

The role of severity of GH deficiency on clinical and instrumental features and response to treatment in children

Solntsava Anzhalika , Zagrebaeva Olga , Mikhno Hanna

Aim: To determine the response to treatment, clinical and instrumental features in children with partial GH deficiency (pGHD) and severe GHD (sGHD).Methods: We examined retrospectively 30 children with isolated GHD (stage on Tanner 1) in the Endocrinological department of University hospital (Minsk) over 2004–2012 years. Group 1 (G1) – children with pGHD (n=5) mean±S.D., age 6.3±1.4 years; group 2 (G2) – s...

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...

ba0006p119 | (1) | ICCBH2017

Phenotypic spectrum in Weyers acrofacial dysostosis: A case report

Rubino Chiara , Stagi Stefano , Petrolini Chiara , Gioe Daniela , La Spina Luisa , Peluso Francesca , Della Monica Matteo , de Martino Maurizio

Background: Weyers acrofacial dysostosis (WAD, OMIM 193530) is a rare autosomal dominant disease, characterized by mildly short stature, postaxial polydactyly, nail dystrophy and dental anomalies. WAD should be distinguished from Ellis-van Creveld syndrome (OMIM 225500), a similar but more severe disease, comprising chondrodysplasia, orofacial anomalies and, in a proportion of patients, cardiovascular malformations. Both diseases are caused by mutations in either EVC or EVC2<s...

ba0005p10 | Arthritis and other joint diseases: translational and clinical | ECTS2016

Identifying cell populations coupling inflammation to osteoresorption in arthritis

Mosler Elvira Lazic , Flegar Darja , Sucur Alan , Fadljevic Martina , Kelava Tomislav , Markotic Antonio , Ivcevic Sanja , Zrinski-Petrovic Katerina , Katavic Vedran , Grcevic Danka , Kovacic Natasa

Rheumatoid arthritis (RA) is characterised by periarticular bone destruction leading to permanent disability. Some other forms of arthritis, such as arthritis in systemic lupus erythematosus (SLE), rarely produce bone lesions. Corresponding to that, inactivation of Fas produces a murine SLE-like phenotype, but alleviates bone loss during antigen-induced arthritis (AIA). Aim of this study was to identify cell populations differentially regulated in non-resorptive vs. resorptive...

ba0001pp269 | Genetics | ECTS2013

Expression analysis of mesenchymal KS483 cells during differentiation towards osteoblasts

Fijalkowski Igor , Boudin Eveline , Borra Vere , Van Hul Wim

The murine osteoprogenitor cell line, KS483 (Percuros, The Netherlands) is a well-established model for investigation of osteoblast differentiation and bone formation processes. The mesenchymal characteristics of this cell line allow it to differentiate into either adipocytes or mature, mineralizing osteoblasts. Various phases can be distinguished during osteoblast differentiation and maturation; namely proliferation, matrix formation, matrix maturation, and mineralization.</p...

ba0001pp303 | Muscle, physical activity and bone | ECTS2013

The effect of different exercise modes on bone density in middle-aged and older men: a systematic review

Bolam Kate A , van Uffelen Jannique G Z , Taaffe Dennis R

Although trials have shown that exercise has positive effects on bone mineral density (BMD), not all exercise modalities are osteogenic and the majority of exercise trials have been conducted in older women. The aim of this study was to systematically review trials examining the effect of weight-bearing and resistance-based exercise modalities on the BMD of hip and lumbar spine of middle-aged and older men. Eight electronic databases were searched in August 2012. Only randomis...