Searchable abstracts of presentations at key conferences on calcified tissues

ba0004op11 | (1) | ICCBH2015

Optimal dose of calcium for treatment of nutritional rickets

Thacher Tom , Smith Lauren , Fischer Philip , Cha Stephen , Isichei Christian , Pettifor John

Objective: Nutritional rickets in Nigerian children is primarily due to dietary calcium deficiency and heals with calcium supplementation. Our aim was to determine the optimal dose of calcium for treatment of children with rickets due to dietary calcium deficiency.Methods: At the Jos University Teaching Hospital, 65 Nigerian children with radiographically-confirmed rickets were randomized to daily supplemental calcium intake of 500 mg (n=21), 10...

ba0004p49 | (1) | ICCBH2015

Using high-resolution peripheral quantitative computed tomography (HRpQCT) to better understand the skeletal response to exercise

Edwards Lauren , Skerry Tim , Paggiosi Margaret , Offiah Amaka

To use HRpQCT to investigate the effects of short term but intense exercise on the bone architecture of the distal radius in exercise-naïve women, with the ultimate aim of developing exercise regimes for children that will maximise their peak bone mass.We have recruited 16 of 20 proposed exercise-naïve women, aged 18–25, for a 12-week exercise study. The exercise consists of supervised hammering of a metal plate, using their dominant arm, ...

ba0007p211 | (1) | ICCBH2019

Value of osteogenesis imperfecta clinical nurse specialists to families and consultants across five UK centres

Heathfield Mark , Tucker Ian , Sahota Jaskiran , Rayner Lauren , Greenacre Gemma

Objectives: To ascertain if parents and consultants at secondary care level hospitals felt there were areas that the Clinical Nurse Specialists (CNS), working with children and families with Osteogenesis Imperfecta (OI) could improve upon within their service, and to gain feedback on the current service provided.Methods: A SurveyMonkey© questionnaire was created through the audit team at Great Ormond Street Hospital and the Paediatric Ost...

ba0006oc10 | (1) | ICCBH2017

Inadequate vitamin D status adversely affects trabecular bone mineral density in 14–18 year old adolescents

Smith Taryn , Tripkovic Laura , Damsgaard Camilla , Molgaard Christian , Hennessy Aine , Dowling Kirsten , Cashman Kevin , Kiely Mairead , Lanham-New Susan , Hart Kathryn

We have previously shown a high prevalence of vitamin D inadequacy (serum 25-hydroxyvitamin D (S25(OH)D) <50 nmol/l) in adolescents (14–18 years) in the UK (51°N)(1). It is well recognised that vitamin D deficiency (S25(OH)D <25 nmol/l) increases the risk of rickets and impaired growth in adolescents, however the optimal vitamin D status for bone health is debated. The aim of this study was to investigate the effects of vitamin D status on bone hea...

ba0002p133 | (1) | ICCBH2013

Linear growth over 2 years of velaglucerase alfa therapy in children with type 1 Gaucher disease previously treated with imiglucerase

Zimran Ari , Hughes Derralynn , Elstein Deborah , Smith Laurie , Harmatz Paul , Rhead William , Giraldo Pilar , Mendelsohn Nancy , Park Chan-Hoo , Zahrieh David , Crombez Eric

Objectives: As children with confirmed type 1 Gaucher disease (inherited metabolic disorder) may have linear growth retardation, we evaluated linear growth over 2 years in children enrolled in the interventional study TKT034, in which patients receiving imiglucerase enzyme replacement therapy were switched to velaglucerase alfa.Methods: Trial TKT034 enrolled patients who were ≥2 years of age with type 1 Gaucher disease and stable clinical parameter...

ba0005p20 | Biochemical testing | ECTS2016

Frozen for 7 years: How long can bones be stored prior to biomechanical testing?

Varela Aurore , Smith Susan

Biomechanical strength testing of bones, considered a key component in bone quality assessment, is a critical end-point in the evaluation of safety and efficacy of test compounds in preclinical studies. Bones are usually preserved frozen and tested within a few months following harvesting. They can also be stored for longer periods and only tested when additional information is required. The objective of this study was to evaluate if differences in biomechanics data between di...

ba0002op11 | (1) | ICCBH2013

Artistic gymnasts display micro-architectural advantages over trampolining and tumbling gymnasts: a high resolution peripheral quantitative computed tomography study

Burt Lauren A , Stanger Scott P , Schipilow John D , Boyd Steven K

Participation in high impact sports during growth increases bone quality. Gymnasts have previously displayed increased bone mass and strength at both the upper and lower limbs compared with controls. However, it is not yet understood how bone microarchitecture is affected by gymnastics participation, and if this differs based on gymnastics discipline. Therefore, the objective of this study was to investigate the influence of gymnastics discipline on bone microarchitecture in a...

ba0004oc8 | (1) | ICCBH2015

Combination sclerostin antibody and zoledronic acid treatment outperforms either treatment alone in a mouse model of osteogenesis imperfecta

Munns Craig , Peacock Lauren , Mikulec Kathy , Kneissel Michaela , Kramer Ina , Cheng Tegan , Schindeler Aaron , Little David

Introduction: Bisphosphonate treatment in children with osteogenesis imperfecta reduces bone catabolism and relies on modelling to form new bone. An anabolic treatment, anti-sclerostin antibody (Anti-SOST Ab), is being investigated in clinical trials. We hypothesized that combined treatment may produce superior outcomes in OI.Methods: Female Col1a2 G610C mice and their wild type littermates (WT) were treated from week 5 to week 9 of life with either sali...

ba0006oc21 | (1) | ICCBH2017

Scoliosis in fibrous dysplasia/McCune-albright syndrome

Berglund Jason , Tella Sri Harsha , Kim Lauren , Stanton Robert , Collins Michael , Boyce Alison

Objectives: Fibrous dysplasia is a rare bone and endocrine disorder resulting from somatic activating mutations in GNAS. In the skeleton, proliferation of undifferentiated stromal cells results in osseous lesions that are prone to deformity, fracture, and pain. Lesions may affect one bone or many, and may occur in isolation or in association with hyperfunctioning endocrinopathies, termed McCune-Albright syndrome (MAS). Scoliosis is a potentially serious, even lethal c...

ba0007oc11 | (1) | ICCBH2019

Targeting adeno-associated viral vectors to fractures and the skeleton

Lee Lucinda , Peacock Lauren , Lisowski Leszek , Little David , Munns Craig , Schindeler Aaron

Objectives: While local gene therapy for bone applications has shown some success in preclinical models, systemic delivery of transgenes to the skeleton remains a considerable challenge. Viral vectors such as adeno-associated viruses (AAVs) have great potential as vectors for systemic transgene delivery and may be adapted for emerging gene editing technologies. Furthermore, AAV vectors can have high efficiency, low immunogenicity, and selective tropism towards different tissue...