Bone Abstracts

Background: Rett Syndrome (RS) is a disabling condition due to mutations in MECP2. Girls affected with RS are at risk of developing osteoporosis and fractures at a young age because of their lack of mobility and through a direct effect of MECP2 on bone mineralization. In these girls, bone fragility inflicts pain and may seriously impair the quality of life.Objective: To retrospectively assess the effect of IV bisphosphonates on fracture, bone mineral den...

Introduction: Administration of antibodies that neutralize sclerostin has been demonstrated to increase bone mass. We report the key findings of a Phase 2 study of the human sclerostin antibody, blosozumab (bmab).Methods: Study GSDB was a randomized, parallel-design, double-blind placebo-controlled study, designed to assess the dose–response relationship of bmab in postmenopausal women with low bone mineral density (BMD; lumbar spine (LS) T...

Osteogenesis imperfecta (OI) type V is caused by a unique dominant mutation (c.−14C>T) in IFITM5, which encodes BRIL, a transmembrane ifitm-like protein most strongly expressed in osteoblasts, while type VI OI is caused by recessive null mutations in SERPINF1, encoding pigment epithelium-derived factor (PEDF). We identified a 25-year-old woman with severe OI, whose dermal fibroblasts and cultured osteoblasts displayed minimal secretion of PEDF, but ...

Muscle and loading force application alters bone structure and increases bone mineral density (BMD), particularly during growth. However, bone microarchitectural texture, as assessed by the trabecular bone score (TBS), changes during growth is unknown. We hypothesized that TBS would be positively correlated with growth and higher in growing girls participating in regular physical exercise.68 girls (mean age 12±0.3 years; BMI 18±2.8 kg/m2</...

Background: Alzheimer’s dementia (AD) and osteoporosis (OP) are common and parallel ageing and frequently coexist in an ageing population. Low BMD appears related to increased risk of AD. Various clinical conditions have been shown to alter acetylcholine (Ach) signalling and affect bone. Ach receptor (AchR) subunits and Ach esterase (AchE) are expressed in bone. Presynaptic Botox inhibit Ach release and impair bone healing and decrease bone mineral content. Poliomyelitis ...

see PP180....

Denosumab (DMAb) has a unique profile of bone resorption inhibition: CTX decreases rapidly by 3 days and inhibition is released at the end of the 6-month dosing interval, when DMAb serum levels decrease (McClung NEJM 2006). The dynamic CTX inhibition profile is not curtailed by continued treatment. In the 3-year FREEDOM study, CTX values at 6 months were influenced by baseline CTX values and days since the 1st injection (Eastell JBMR 2011). With 3 additional ...

HLA-B27 transgenic rats, developed by inserting into rat genome the gene for HLA-B27, a human Class I major histocompatibility molecule involved in antigen presentation, spontaneously develop arthritis, ankylosing spondylitis, gastrointestinal inflammation, and severe alveolar bone loss, among other signs of a generalized inflammatory response. Clinical manifestations in these rats closely resemble features of HLA-B27–associated diseases in humans. More recently, investig...

Background: Osteoporosis is not a homogenous disease. Riggs et al. identified two distinct types of osteoporosis, with different pathophysiology, patterns of bone loss and fracture types.Post-menopausal (PM) osteoporosis is triggered by withdrawal of the effect of oestrogen on bone, which leads to a sharp acceleration of bone turnover with an imbalance towards excessive osteoclastic activity. Senile osteoporosis in the old old (usually after the...

Objectives: Most osteolytic tumors are in fact mixed and contain an osteoblastic component associated with the predominant osteolytic areas. This metaplastic woven bone can be evidenced on X-rays as blurred white areas but is always evidenced by histological analysis even in the absence of radiological expression. Metaplastic bone formation reflects the activation of new osteoblasts, the stimulation of the dormant lining cells due to the release of growth factors buried in the...