Bone Abstracts

Steroids are currently used to improve muscle strength and prolong ambulation in boys with Duchenne muscular dystrophy (DMD) although the effect on bone health is still unclear. The aim of this study was to compare bone strength in healthy children and boys with DMD and investigate the interaction between diminished muscle function, loss of ambulation and high dose oral steroids.Fifty children were studied, 14 healthy boys (HB), 13 boys with DMD who rema...

Vertebral fracture assessment (VFA) by DXA is an accepted tool in adults. However, its use in children has not been validated. The aim of this study was to validate VFA using iDXA against spinal radiographic assessment (RA) for the identification of vertebral fractures in children.Spine radiographs and VFA (L5–T2) by GE-iDXA were acquired on the same day in 80 children. Forty children were considered high-risk for fracture as their metabolic bone sp...

Introduction: Despite the popularity of platet lysate (PL) treatments in orthopaedics, the mechanism of action and the effectiveness of this therapeutic tool is still controversial. So far, the activity of PL has been associated with different growth factors (GFs) released upon platelet degranulation. However, PL activity might also be due to the efficient cell to cell transport system of GF and other bioactive molecules by their encapsulation into exosomes. In this study, we ...

Proteoglycans is a component of extracellular matrix, forming a mesh around structural proteins including collagen and elastin, and providing elasticity to tissue structure. Proteoglycans is formed by the attachment of glycosaminoglycans to a core protein, a process that requires three enzymes: galactosyltransferase-1, galactosyltransferase-2 and glucoronosyltransferase-1, encoded by B4GALT7, B3GALT6 and B3GAT3 respectively. B3GALT6 mutation...

Intravenous (IV) Pamidronate (PAM) has been used in the treatment of Osteogenesis Imperfecta (OI) and is known to increase bone mineral density (BMD) and reduce the incidence of fractures. However an attractive alternative is the more potent IV Zoledronic acid (ZOL).Objectives: To determine the clinical efficacy of IV PAM vs ZOL in children with mild to moderate OI and compare the cost benefits of the two drugs.Methods: A retrospec...

Introduction: Since the definition of osteoporosis in children changed following the revised ISCD guidelines of 2013, the presence of vertebral fractures has become more clinically important, in particular since vertebral fractures may occur despite apparently normal bone density (www.iscd.org). Although the detection of vertebral fractures in children is still largely dependent on lateral spine x-rays, the introduction of new higher resolutio...

Osteosarcoma (OS) is an aggressive malignancy characterized by a high relapse rate despite multiagent chemotherapy. As for other cancers, OS microenvironment contributes to tumor initiation, growth, and metastasis. We consider bone marrow mesenchymal stromal cells (MSC) as a major nontumor component of OS microenvironment, and have previously found that the interaction between MSC and tumor cells is bidirectional, i.e. tumor cells can modulate their peripheral environment that...

Molecularly characterized epigenetic networks that control bone formation and are altered during aging are necessary to uncover new potential targets for osteoanabolic therapy. Our contribution to the iBONE consortium is to identify osteoanabolic epigenomic regulators by screening which are involved in osteoblast phenotype and differentiation. This study will be done by a 3 step approach including: siRNA screening for epigenomic regulators of osteoblastic differentiation and v...

Objectives: The SNAP study is a prospective fracture study of children with chronic inflammatory and/or disabling conditions. The overall study aim is to assess causal links between body-size adjusted bone density and low trauma fracture.Methods: 330 children aged 5–18 years were recruited from seven disease groups namely; acute lymphoblastic leukaemia (ALL), rheumatological disease, inflammatory bowel disease, cystic fibrosis, coeliac disease, Duch...

Objectives: Current guidelines recommend annual assessments of bone densitometry in boys with Duchenne muscular dystrophy (DMD). However, this recommendation is based on the assumption that bone density is a predictor of fractures in this patient group. The aim of this study was to evaluate the relationships between long-term changes in bone density, corticosteroid exposure and mobility with vertebral and long bone fractures.Methods: Twenty-four DMD boys...