Bone Abstracts

Acute phase reaction (APR) is the most frequent side effect following i.v. nitrogen-containing bisphosphonates (N-BPs) infusion. A recent observation in osteoporotic women treated with N-BPs evidenced a negative association between 25(OH)D levels and APR, likely due to the immuno-modulatory effects of vitamin D on γδTcells. However, this association remains to be demonstrated in patients with Paget’s disease of bone (PDB). Moreover whether vitamin D supplementat...

Defects in 24-hydroxylation caused by vitamin D-hydroxylase (CYP24A1) loss-of-function mutations lead to decreased degradation of 1,25(OH)2D and the syndrome of idiopathic infantile hypercalcemia. Affected individuals show increased sensitivity to vitamin D and may develop severe hypercalcemia and hypercalciuria, even with small doses of vitamin D.Presenting: The objective of the study was to investigate the gene expression profile in...

Pathophysiological calcification in the vasculature favours cardio- and cerebrovascular diseases. In patients with chronic kidney disease vitamin K metabolites are associated with decreased vascular calcification.We investigated the expression of vitamin K dependent proteins (VKDPs) in vessels and bone to identify differences in expression pattern during atherosclerosis (AS) stages and compare the two tissue profiles.Gene expressio...

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...

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...

Periostin (osteoblast-specific factor OSF-2) is a glycoprotein that in humans is encoded by the POSTN gene. It is a component of the extracellular matrix and is thought to be involved in osteoblast recruitment, attachment and spreading.The N-terminus part of periostin is conserved, while the C-terminal region gives rise to different splice isoforms upon alternative splicing. The isoforms have a molecular weight range from 83 to 93 kDa.<p class="abste...