Bone Abstracts

Aim: Scoliosis is a common skeletal problem affecting 10–30% of patients with neurofibromatosis type 1 (NF1). NF1 patients have been shown to have reduced bone mineral density (BMD) which may play a role in the pathogenesis or progression of scoliosis. Our centre is one of four international centres currently evaluating the efficacy of various spinal imaging techniques and BMD as predictors for scoliosis in NF1. In our cohort we measured the lumbar spine (LS) BMD both by ...

Objective: Bone and muscle deficits are observed in patients with Crohn’s disease (CD). High-impact exercise (HIE), such as jumping based exercise, can provide hypertrophic and osteogenic stimulus, however to date there have been no studies of HIE in CD. This study aimed to assess the acceptability and feasibility of participating in HIE in adolescents and adults with CD.Methods: Two anonymous questionnaires surveyed adolescents and adults, respecti...

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...

Background: We discovered that variants in NBAS (Neuroblastoma Amplified Sequence Gene) known to be associated with acute liver failure are also responsible for skeletal abnormalities. This work was published as a novel cause of bone fragility [Balasubramanian et al., 2017]. NBAS role in bone fragility provides an opportunity to use tractable animal research to advance understanding of mechanism and identify potential new treatments. This would be beneficial to patien...

Introduction: Nitrogen-containing bisphosphonates (N-BPs) can inhibit the differentiation and function of osteoclasts derived from Peripheral Blood Mononuclear cells (PBMCs) in a dose-dependent manner. MUTZ-3 cells are a potentially useful human cell line for studying osteoclast differentiation. The aim of this study was to elucidate the action of N-BPs on MUTZ-3 cells.Methods: Human PBMCs and MUTZ-3 cells were cultured in α-MEM supplemented with he...

Cystic fibrosis(CF) results in low volumetric bone mineral density(vBMD), poor muscle strength and increased fracture risk in young patients. The aim of this study was to compare bone and muscle variables measured by peripheral and high-resolution QCT (pQCT and HR-pQCT) and jumping mechanography (JM) In CF children and healthy controls. We hypothesised that CF children have lower muscle force and power (Fmax and Pmax) than controls which may contribute to CF-related bone disea...

Objectives: To evaluate musculoskeletal development using pQCT and DXA in childhood onset inflammatory bowel disease (IBD).Methods: Prospective longitudinal study with 12 months follow-up in 43 children (23 males) with IBD: 30 crohn’s disease(CD), 13 ulcerative colitis (UC) and inflammatory bowel disease unclassified (IBDU). pQCT at 4% and 66% radius, DXA for total body (TB), lumbar spine (LS) bone mineral content were assessed at baseline and 12 mo...

Objective: To evaluate bone and muscle mass in children with inflammatory bowel disease (IBD) following infliximab (IFX) therapy.Methods: Prospective longitudinal study of 19 children (12 males), 17 crohn’s disease (CD), 1 ulcerative colitis (UC), 1 IBD unclassified (IBDU) of bone evaluation commencing treatment with IFX. Bone and muscle parameters were measured by pQCT at the non-dominant distal radius at 4 and 66% at baseline and 6 months. pQCT pa...

Type V OI is characterised by interosseous membrane calcification and hyperplastic callus formation, but the infantile phenotype is less well recognised. In 2012 Arundel et al. described distinctive radiographic changes in an infant with type V OI. We report two further male infants (with genetic confirmation of type V OI) confirming the highly characteristic and consistent radiographic appearances that should aid early diagnosis.Case 1 – P...

Background: Anorexia nervosa (AN) presenting in childhood can have devastating implications for growth, puberty, menstruation and bone health. It may lead to altered bone structure and inadequate acquisition of bone mass with increased risk of fracture.Objective: To describe growth, pubertal and bone mineral density data in a cohort of adolescents with AN managed in a tertiary metabolic bone service.Methods: 62 adolescent females w...