Bone Abstracts

Objectives: To determine if genetic variants associated with principal component-derived areal bone mineral density (aBMD) loading scores.Methods: Our sample comprised 1,293 children of European ancestry enrolled in the longitudinal Bone Mineral Density in Childhood Study (52% female). The participants completed up to 7 annual study visits. From dual energy X-ray absorptiometry scans, sex and age-specific aBMD Z-scores were calculated for total hip, femo...

Objective: The forearm is a common fracture site during childhood, but DXA pediatric reference data for areal bone mineral density (aBMD) at the ultradistal (UD) radius are lacking. The objective of this study was to first, develop age-, sex-, and ancestry-specific reference data for UD radius aBMD; second, assess the relationship between UD radius aBMD and a) other DXA aBMD measures and b) radius bone volumetric density and geometry by pQCT; and third, examine the tracking of...

Objective: With recent genome wide association studies (GWAS), ~70 loci have been robustly and reproducibly associated with adult bone density and/or osteoporosis. However, to date no systematic effort has investigated which of these loci operate early in life. We investigated whether these single nucleotide polymorphisms (SNPs) are associated with childhood areal bone mineral density (aBMD). In addition we determined if any of the associations were age dependent.<p class=...

Objectives: To compare and contrast the natural history of osteoporosis and response to zoledronate in children and adolescents with Duchene muscular dystrophy (DMD), spinal muscular atrophy (SMA) or other congenital muscular dystrophies (CMD).Methods: A retrospective medical record review of fracture history, treatment and bone mineral densitometry of children managed at a tertiary centre in Sydney over the last 6 years.Results: A...

Objective: Vascular calcification contributes to the pathogenesis of atherosclerosis, hypercholesterolemia, end stage renal disease, and diabetes, and is caused by genetic ablation of matrix Gla protein (MGP) in mice. Genetic ablation of MGP in mice results in excessive calcification of the vessel wall associated with a chondrocyte-like trans-differentiation in smooth muscle cells (VSMCs). Canonical β-catenin signaling is activated in the calcified arterial Mgp&#8722...

Background: Denosumab (DMAB) is used in adults for the treatment of osteoporosis, giant cell tumour of bone, and cancer metastases. There are little data on paediatric use with clinical decision making reliant on adult data and clinical experience.Presenting problem: We have treated 33 children with DMAB: Perthes disease (n=9), avascular necrosis (n=17), osteoporosis (n=1), aneurysmal bone cyst (n=4) and giant cell tum...

Objectives: Clinicians practicing in long-term care (LTC) face unique challenges caring for frail elderly individuals including multiple co-morbidities, polypharmacy, and end of life care, and practice guidelines typically do not address this population. Guidance regarding the management of osteoporosis and fracture prevention in LTC, a high-risk population, is needed.Materials and methods: A survey of LTC physicians informed key questions and outcomes, ...

Introduction: Epithelioid hemangioendothelioma (HEE) is a tumor of vascular origin, infrequent in the pediatric age and even more infrequent at intestinal level. To our knowledge, there are no previous reports of pediatric patients with malignant humoral hypercalcemia associated with this tumor. Humoral mechanism is seen more often in lung, uterine cérvix, skin and esophagus tumors. The presence of hypercalcemia appears to be an ominous prognostic sign.<p class="abste...

Objectives and methods: Exercise induces greatest bone gains during growth, yet reduced bone strength is an age-related phenomenon. This raises the question of whether exercise-induced bone changes when young persist into adulthood. Previous work has suggested exercise-induced gains in bone mass are lost with aging. However, exercise during growth primarily influences bone structure rather than mass to increase strength and mechanisms exist for the maintenance of exercise effe...

Objective: The physis, or growth plate, is comprised of precisely organized chondrocytes that confer longitudinal growth of the bone. Multiple signaling pathways cooperate to regulate growth through their control of chondrocyte shape, polarity, proliferation, and differentiation.1,2 Disruption of these cellular events result in physeal defects, skeletal deformities, and abnormal limb growth. Loss of function mutations in Smad4, a common intracellular effector of all...