Searchable abstracts of presentations at key conferences on calcified tissues

ba0006p146 | (1) | ICCBH2017

Stature and longitudinal growth in glucocorticoid naive boys with Duchenne Muscular Dystrophy

Joseph S , Edwards G , DiMarco M , Abu-Arafeh I , Baxter A , Horrocks I , McWilliams K , Naismith K , Stephen E , Ahmed S F , Wong S C

Background: Previous studies with small number of boys with Duchenne Muscular Dystrophy (DMD) suggest that growth failure occurs in glucocorticoid naïve (GC) boys.Objective: To evaluate height and longitudinal growth in boys with DMD prior to GC.Method: Retrospective evaluation in boys with DMD with height measurements obtained for clinical purposes. Out of the 91 boys currently managed in Scotland, 51 had at least one height ...

ba0007p71 | (1) | ICCBH2019

Necessity of high dose and prolonged duration denosumab post stem cell transplant for TNFRSF11A osteoclast-poor autosomal recessive osteopetrosis

Taylor-Miller Tashunka , Doss Hemalatha , Weerdenburg Heather , Whiting Sam , Sivaprakasam Ponni , Gassass Adam , Smithson Sarah F , Steward Colin G , Burren Christine P

Background: Hypercalcaemia is a risk following stem cell transplant (SCT) for all types of autosomal recessive osteopetrosis (ARO) due to restored osteoclast differentiation. This can be particularly severe in the osteoclast-poor (OP) form involving the tumour necrosis factor receptor superfamily 11A (TNFRSF11A) gene, encoding RANK. Denosumab, a monoclonal antibody blocking RANK activation, has been described for refractory post-SCT hypercalcaemia in two cases. Our case adds n...

ba0001pp393 | Osteoporosis: treatment | ECTS2013

Remarkable bone mineral density increases on teriparatide in patients with glucocorticoid-induced osteoporosis and Crohn's disease

Ko-Wu Kuo Danny , To Kenny , Kendler David

Crohn’s disease often results in abnormalities in bone strength, and ultimately increases the risk of fragility fracture. Up to 55% of patients with Crohn’s disease have bone mineral density in the osteopenia range up to 50% of osteoporosis. Glucocorticoid is frequently used in the treatment of Crohn’s disease and is associated with osteoporosis and increased fracture risk. It has been reported that osteoporotic fractures in patients with Crohn’s disease ar...

ba0005p185 | Cell biology: osteoclasts and bone resorption | ECTS2016

Characterising the role of the lysosomal membrane proteins MFSD1 and TMEM106b in osteoclasts

Lopez David Massa , Damme Markus , Saftig Paul

Osteoclasts are highly specialized cell types, responsible for the resorption of bone matrix. Coordinated with osteoblasts they contribute to a proper bone turnover. An impaired or reduced function of the osteoclast leads to a pathogenic increase of the bone mass and finally osteopetrosis. Lysosomal hydrolases, as exemplied by CTSK and Acp5 (TRAP), are known to play an important role in the function of osteoclasts, and knockout mouse models of these proteins develop a bone res...

ba0005p390 | Osteoporosis: treatment | ECTS2016

Denosumab therapy results in a high frequency of responders by bone mineral density in both treatment-naïve patients and patients switching therapies

Almohaya Mohammed , Liu Angela , Kendler David

Clinical trials suggest that denosumab (DEN) therapy results in greater increases in bone mineral density (BMD) in treatment-naive patients than in patients switched from bisphosphonates.We retrospectively reviewed charts of all patients treated with DEN at an osteoporosis referral centre in Vancouver, Canada including all patients treated with DEN 60 mg SC every 6 months for 1 year or more, and in whom baseline and follow-up BMDs were available. BMD was...

ba0002p37 | (1) | ICCBH2013

Reduction in bisphosphonate side effect profile using short-term steroid cover

Biggin Andrew , McLean Tina , McQuade Mary , Cowell Chris , Munns Craig

Objectives: Bisphosphonate infusions are associated with numerous adverse effects including acute systemic inflammatory reactions and electrolyte abnormalities. The aims of this study were to evaluate the safety and efficacy of a 3-day course of prednisone on children receiving their first dose of pamidronate or zoledronic acid.Methods: A retrospective cohort of 166 patients (85 males) were commenced on pamidronate (16%) or zoledronic acid (84%) for trea...

ba0005p342 | Osteoporosis: pathophysiology and epidemiology | ECTS2016

Short-term smoking cessation improved bone formation in healthy male smokers

Watanabe Reiko , Tai Nobuyuki , Hirano Junko , Masaki Hiroaki , Okazaki Ryo , Inoue Daisuke

Background and Aim: Smoking increases fracture risk and contributes to COPD-associated osteoporosis. However, its impact on bone metabolism is largely unknown. We thus aimed to determine the effect of smoking cessation on bone metabolism.Subject and Method: In this prospective study, we recruited 29 healthy Japanese male subjects with smoking habit (37.7±8.2 years old, 16.9±11.3 pack years). Pulmonary function test was done before smoking cessa...

ba0002p196 | (1) | ICCBH2013

Bigger but not stronger? GH treatment in Turner syndrome may confer no benefit to HR-pQCT determined bone micro-architecture

Nour Munier , Boyd Steven K , Perry Rebecca J , Stephure David K , Hanley David A

Turner syndrome (TS) is known to be associated with increased risk of osteoporosis and fracture. Childhood treatment with GH has been considered standard of care for treatment of growth failure in TS, while the influence of GH on bone health has been poorly understood. The purpose of this study is to assess the influence of GH on bone microarchitecture on a cohort of TS subjects.TS subjects aged 16–45 were included. Bone mineral density (BMD) was as...

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...