Bone Abstracts

Glucocorticoids (GCs) are widely used to treat chronic inflammatory diseases such as rheumatoid (RA) and lead to multiple side effects including glucocorticoid induced osteoporosis (GIO). Our work challenges the dogma that transrepression of pro-inflammatory genes by the glucocorticoid receptor (GR) is solely responsible for reducing inflammation, whereas transactivation of genes is causing side effects.Using conditional and function selective mutant mic...

The chronic use of glucocorticoids in patients with congenital adrenal hyperplasia (CAH) may result in decreased bone mass. Therefore, using simple and accurate methods for assessing bone status in these patients could facilitate the treatment of disease. The purpose of this study was to verify the association between parameters of bone mass measured by dual energy X-ray absorptiometry (DXA) and quantitative ultrasound (QUS) of proximal phalanges in children adolescents with c...

Department of Biomedical Sciences and Medicine and Centre of Marine Sciences, University of Algarve, Faro, PortugalIn the last decade there has been a growing interest towards the use of fish as models to understand the basic mechanisms of cartilage and bone formation, maintenance and regeneration. In particular, zebrafish and medaka have become accepted models for human skeletal development and associated pathologies such as craniofacial dysplasia, oste...

Acute lymphoblastic leukemia (ALL) is the most frequent form of childhood malignancy, with a cure rate that now exceeds 80%. As survival improves, the clinical consequences of ALL and its treatment are increasingly recognized, with skeletal health emerging as an important care issue. The skeletal morbidity that arises in this setting falls into two main categories: osteoporosis (low trauma fractures) and osteonecrosis (in situ bone death). Osteoporosis and necrosis ha...

Additionally to the standard histomorphometric and pathohistologic evaluation, the transiliac bone biopsy sample from a patient can be further analyzed for additional information on structural and material characteristics. Under the term ‘new histology’, several non-destructive techniques and combinations thereof providing high spatial resolution have been introduced. In contrast to conventional histology, these techniques allow the characterization of structural, co...

Osteoporosis in Duchenne Muscular Dystrophy (DMD) is arguably one of the most severe bone fragility conditions among children with chronic illnesses. This is hardly surprising, given the deleterious effects of the myopathy and glucocorticoid (GC) therapy on bone strength. The severity of the osteoporotic phenotype is highlighted by observations that 60% of boys will sustain long bone fractures during childhood and a third will present with back pain due to vertebral fractures ...

Biographical DetailsLeanne WardDr Leanne Ward is an Associate Professor of Pediatrics at the University of Ottawa where she has held a Research Chair in Pediatric Bone Health since 2010. She is the Medical Director of the Pediatric Bone Health Clinical and Research Programs at the Children’s Hospital of Eastern Ontario. Dr Ward’s research ...

Intravenous (IV) bisphosphonates (pamidronate, zoledronic acid and neridronate) are the mainstays of medical therapy for numerous pediatric bone diseases. While most frequently prescribed for hypercalcemic disorders and osteoporosis, their potent analgesic effects have also led to their use in fibrous dysplasia, osteonecrosis, sickle cell disease, chronic non-bacterial osteomyelitis, and complex regional pain syndrome. Bisphosphonates given IV are highly effective in preventin...

The role of the WNT pathway in skeletal maintenance has been extensively studied since the identification of mutations in key signaling WNT mediators (LRP5 and sclerostin) in high and low bone mass phenotypes. However, the identity of the key WNT ligand that signals via LRP5/6 has remained unknown. We aimed to identify genes with a major effect on the skeleton by studying individuals and families with early-onset osteoporosis or osteogenesis imperfecta (OI).<p class="abste...

Objectives: Osteopenia is a complication of childhood cancer treatment but it is unknown to which extend this occurs in adult survivors, and which subgroups are at risk. We examined bone mineral density (BMD) and assed the relative importance of potential determinants of low BMD in very long-term adult survivors of childhood cancer.Methods: The single-center cohort study included 410 adult CCSs (median age at diagnosis: 6.6 years (range: 0–17 years)...