Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p100 | (1) | ICCBH2019

High-resolution MRI assessment of the muscle-fat-bone unit in young adults with childhood onset Crohn's disease

Steell Lewis , Johnston Blair , Ahmed S Faisal , Gaya Daniel R , MacDonald Jonathan , Seenan John Paul , Russell Richard K , Gray Stuart , Wong Jarod

Objective: Childhood onset Crohn’s disease (CO-CD) is associated with musculoskeletal deficits. However, there are limited data regarding muscle-bone outcomes in adults with CO-CD. The current study aimed to comprehensively assess the muscle-fat-bone unit in young adults with CO-CD, using novel methods of MRI, in comparison with healthy controls.Methods: Trabecular microarchitecture and cortical geometry of the distal femur were assessed using 3T mi...

ba0002p137 | (1) | ICCBH2013

Influence of nutritional status in the bone mass assessed by phalangeal quantitative ultrasound of girls aged 7–10 years old

Bertapelli Fabio , Goncalves Ezequiel M , Barbeta Vinicius J O , Krahenbuhl Tathyane , Ramalho Luis C B , Martin Juan E Samur-San , Ribeiro Roberto R , Guerra-Junior Gil

The childhood obesity is a global epidemic. Some studies with pre-pubertal children reported a positive relationship between fat mass and bone. However, there are no available data on the influence of the nutritional status on parameters of quantitative ultrasound (QUS). The aim of this study was to evaluate the influence of nutritional status in bone mass assessed by QUS of proximal phalanges in girls. The bone mass parameter, amplitude dependent speed sound (AD-SoS) in meter...

ba0003pp159 | Cell biology: osteoclasts and bone resorption | ECTS2014

Arachidonic acid and docosahexaenoic acid inhibits osteoclastogenesis and bone resorption in human CD14+ monocytes, in vitro

Kasonga Abe , Kruger Marlena , Coetzee Magdalena

The mature human skeleton is a metabolically active organ that is continuously resorbed and rebuilt by osteoclasts and osteoblasts. Dietary supplementation of selected long chain polyunsaturated fatty acids (LCPUFAs) has shown effects on bone turnover. Most research on LCPUFAs has been done using commercially available cell lines and further clarification of the cellular effects on models relevant to humans is required. After ethical approval, peripheral blood was collected fr...

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....

ba0001pp82 | Bone development/growth and fracture repair | ECTS2013

Measurement properties of radial and tibial speed of sound for screening bone health and fragility in 10–12 years old boys and girls

Rebocho Lurdes , Cardadeiro Graca , Zymbal Vera , Goncalves Ezequiel M , Sardinha Luis B , Baptista Fatima

The objective of this study was to analyze measurement properties of BeamMed Omnisense quantitative ultrasound (QUS) of the radial and tibial speed of sound (SoS) for assessing bone health and screening bone fragility in youth. Bone fragility was defined as low whole body less head bone mineral density (WBLH BMD) measured by DXA (first tertile, 95% CI: −1.1– (−0.9)) and as past history of fractures evaluated by questionnaire. The study was conducted with 319 n...

ba0004p105 | (1) | ICCBH2015

Refractory hypercalcaemia of malignancy: responsiveness to Denosumab and Zoledronate

Giri Dinesh , Ramakrishnan Renuka , Hayden James , Brook Lynda , Das Urmi , Mughal M Zulf , Selby Peter , Dharmaraj Poonam , Senniappan Senthil

Background: Hypercalcaemia secondary to malignancy is rare in children and adolescents. Parathyroid hormone related peptide (PTH-rP) secreted by malignant cells increases bone resorption and renal calcium retention causing hypercalcaemia. We report two cases of hypercalcaemia of malignancy refractory to treatment with pamidronate and corticosteroids but responsive to treatment with Denosumab and Zoledronic acid.Case 1: Presenting problem: A 17-year-old b...

ba0002p181 | (1) | ICCBH2013

Long-term imiglucerase/alglucerase treatment in Latin American children with type 1 Gaucher disease: lessons from the International Collaborative Gaucher Group (ICGG) Gaucher Registry

Camelo Jr Jose Simon , Cabello Juan Francisco , Drelichman Guillermo G , Kerstenetzky Marcelo M , Sarmiento Isabel C , Linares Adriana

Objective: Evaluate the clinical characteristics of all Latin American pediatric patients with Gaucher disease type 1 (GD1) enrolled in the ICGG Gaucher Registry at baseline and investigate long-term outcomes and clinical benefit of prolonged imiglucerase/alglucerase therapy in patients with manifestations of GD1 at baseline.Methods: All Latin American patients with GD1 in the ICGG Gaucher Registry (NCT00358943) who were <18 years of age at the start...

ba0006p104 | (1) | ICCBH2017

Dietary calcium deficiency contributes to the causation of nutritional rickets (NR) in the United Kingdom (UK): data from the British Paediatric Surveillance Unit (BPSU) NR survey

Zulf Mughal M. , Calder Alistair , Blair Mitch , Julies Priscilla , Pall Karina , Lynn Richard , McDonnell Ciara , McDevitt Helen , Shaw Nick J.

Background: Rickets is a disorder of the growing child arising from impaired mineralisation of the growth plate and osteoid. The most common cause of NR in the UK is thought to be secondary to vitamin D deficiency [VDD; serum 25-hydroxyvitamin D (25OHD) <25 nmol/l], although in some African & South Asian countries dietary calcium deficiency (DCaD) by itself, or together with VDD is an important cause of NR (Ann Trop Paediatr. 2006;26:1–16). Currently, the data on ...

ba0005oc3.1 | Clinical trials, FGF-23 and focal osteoporosis | ECTS2016

Effect of odanacatib on bone density and estimated bone strength in postmenopausal women: a CT-based sub-study of the phase 3 long-term odanacatib fracture trial (LOFT)

Langdahl Bente , De Villiers Tobias , Keaveny Tony M , Engelke Klaus , Genant Harry , Ather Shabana , Giezek Hilde , Lombardi Antonio , Leung Albert , de Papp Anne

Odanacatib (ODN), a selective oral inhibitor of cathepsin K, is in development for the treatment of osteoporosis. In the Phase 3, Long-Term ODN Fracture Trial (LOFT; NCT00529373), ODN significantly reduced fracture risk. This imaging sub-study primarily investigated the effect of ODN on volumetric BMD (vBMD) of the lumbar spine (LS) and total hip (TH) using quantitative computed tomography (QCT).Women aged ≥65 without baseline radiographic vertebra...

ba0005p194 | Cell biology: osteoclasts and bone resorption | ECTS2016

Neuropetide Y Y1 receptor deletion impairs matrix demineralization and resorption

Sousa Daniela M , Conceicao Francisco , Leitao Luis , Neto Estrela , Alves Cecilia J , Alencastre Ines S , Herzog Herbert , Aguiar Paulo , Lamghari Meriem

Neuropeptide Y Y1 receptor (Y1R) signalling has been shown to play a key role in bone homeostasis, emerging as a novel therapeutic target in bone diseases. Y1R knockout mice (Y1−/−) display a high-bone mass phenotype that has been mainly attributed to increased osteoblast activity. Nevertheless, the Y1R regulatory role on osteoclastogenesis and matrix resorption remains largely unknown. To clarify th...