Bone Abstracts

Improvements in diagnosis, multi-modal therapy and supportive care over the past several decades have resulted in substantial reductions in mortality rates for childhood cancer. Approximately 80% of children diagnosed with cancer are now expected to survive for at least 5 years after initial diagnosis. Nevertheless, improvements in survival rates have not come without cost, with survivors at risk of skeletal morbidities as a result of disturbances in normal bone metabolism dur...

Biographical DetailsDr C Wilson received a PhD in Epidemiology from the University of New South Wales, Australia, in 2008 for research focusing on the late complications of anti-cancer therapies among individuals diagnosed with childhood cancer. She then worked for a short time as study coordinator for the New South Wales Childhood Cancer Survivor Study before coming to work in the Depart...

The in vitro culture of calvarial osteoblasts from neonatal rodents remains an important method for studying the regulation of osteoblast function. Widespread use of transgenics has created a particular need for a reliable, simple method that allows the differentiation and bone-forming activity of mouse osteoblasts to be studied directly. We have established such a method and have identified key differences in optimal culture conditions between mouse and rat osteoblas...

Background: The significant increase in the life expectancy of the patients with cystic fibrosis (CF) came with some costs, as new complications have emerged. Among endocrine disorders, CF-related bone disease (CFBD) is a leading complications reported in the adult patients.Objectives: Our study present the first results of the bone mass and body composition evaluation by DXA in a small group of Romanian children with CF treated in the author’s depa...

Objectives: X-linked hypophosphataemia (XLH) is a rare, inherited, genetic disease characterised by renal phosphate wasting, bone mineralisation defects, rickets, abnormal tooth development, poor growth and, often, bone pain. Common treatment of children involves supplementation with oral phosphate and active vitamin D (often termed ‘conventional therapy’). The objective of this study was to identify and understand the perceived limitations of conventional therapy fo...

Sclerostin, encoded by the SOST gene, functions as an inhibitor of the Wnt pathway and thus it is an important regulator of bone homeostasis. The fact that osteoblasts, the only cells expressing SOST, lay buried deeply in the bone matrix, poses intrinsic difficulties to the study of the regulation of this gene. Since RUNX2 and SP7/OSX are two known regulators of the differentiation of cells of the osteoblastic lineage, the aim of this study was to determine t...

Individuals with Down syndrome (DS) have a number of phenotypic features, including a short stature. It has been reported that people with DS have lower areal BMD than the general population, but this may be a biased result due to the smaller size of the skeleton, and it is unclear if individuals with DS have fragile bones. Thus, the objective of this study was to explore the skeleton of DS using two techniques, TBS and calcaneal ultrasound, which are not influenced by bone si...

Ecto-nucleotide pyrophosphatase/phosphodiesterases (NPPs) hydrolyse nucleotide triphosphates to the corresponding nucleotide monophosphate and the mineralisation inhibitor, pyrophosphate (PPi). This investigation examined the role of NPP1 in bone and soft tissue mineralisation using a mouse model lacking NPP1(Enpp1−/−). At physiological pH 7.35, cultured Enpp1−/...

Extracellular nucleotides stimulate both the formation and resorptive activity of osteoclasts. Ecto-nucleotide pyrophosphatase/phosphodiesterases (NPPs) hydrolyse extracellular nucleotide triphosphates to their corresponding monophosphate and pyrophosphate (PPi). We investigated if osteoclasts express functional NPPs and whether Enpp1 gene deletion influenced osteoclast formation and activity. Osteoclasts were formed from the bone marrow of 8 and 15 week old knockou...

Aim: The aim of the study was to evaluate the usefulness of the fracture risk evaluated through the FRAX® model based only on the clinical risk factors as a screening tool for identify the target population for treatment in osteoporosis.Materials and methods: Two hundred and seventy-six postmenopausal women treatment naive referred to two different endocrinology departments for osteoporosis between 2009 and 2011 were evaluated. The FRAX® model ...