Bone Abstracts

Objectives: Skeletal abnormalities occur in half of the individuals with neurofibromatosis type-1 (NF1), usually in the first 2 years of life; the most difficult to manage is congenital pseudarthrosis of tibia (CPT, tibial non-union) which may lead to the amputation of the limb. Previous work in our lab identified that high levels of β-catenin early in fracture repair, result in a phenotype similar to CPT. There is data showing higher than normal β-catenin levels in ...

Objectives: To evaluate bone health and its possible correlations to body composition parameters in young patients with galactose metabolic disorders, aiming to suggest appropriate lifestyle interventions.Methods: We studied 22 patients, aged 5–16 years with galactose metabolic disorders, detected by neonatal screening. Fourteen suffered from classic galactosemia and eight from other galactose metabolic disorders (i.e. epimerase or galactokinase def...

Objectives: AIS is associated with both low bone mass and elevated serum bone alkaline phosphatase. The greater the latitude of the geographical region, the higher is the prevalence of AIS. These specific features were compatible with the presence of either Vit-D insufficiency or abnormal physiology with Vit-D. It is important to evaluate these potentially treatable conditions regarding their roles in the etiopathogenesis of AIS. The objectives of this case-control study were ...

Objective: Associations between childhood obesity and disturbed bone metabolism have been extensively studied. Both RANK/RANKL/OPG and adipocytokines are involved in bone metabolism in obese individuals although published data remain inconsistent. Some reports show evidence of protective role of adiposity in the maintenance of skeletal mass, whereas others fail to support this evidence or even demonstrate an increased fragility. The aim of the study was to assess bone metaboli...

Children with chronic medical conditions are at increased risk for bone fragility from multiple mechanisms, related both to the underlying condition and its treatment, in particular glucocorticoids. The differential effects of the underlying medical disease on bone micro-architecture have not been well elucidated.Objectives: To describe the bone micro-architectural characteristics in children with rheumatic and oncological disorders treated with glucocor...

Juvenile Paget’s disease (JPD) is an extremely rare, yet painful and debilitating bone disease with onset occurring during early childhood. JPD can be caused by loss of function of osteoprotegerin, resulting in subsequent osteoclast stimulation via the activated receptor activator of nuclear factor-kappa B (RANK) pathway. Increased bone turnover and a lack of bone modelling lead to severe deformities, frequent fractures, short stature and loss of hearing.<p class="abs...

Antenatal glucocorticoid (GC) injections are not only used to enhance fetal lung maturation in preterm children but also for the treatment of maternal conditions such as autoimmune diseases or infections. Animal models and clinical studies suggest that the regulation of the hypothalamic–pituitary–adrenal axis is altered in the offspring of GC-treated mothers with increased sensitivity to stress.Objective: The aim of this study is i) to define t...

Intermittent PTH administration (iPTH) increases bone mass in humans and animals. PTH exerts its effects by binding to PTH type 1 receptor (PTHR1) predominantly expressed in osteoblasts, resulting in activation of multiple downstream signaling pathways. In vitro investigations have suggested that p38 mitogen-activated protein kinase (MAPK) signaling is an important mediator of PTH-induced osteoblast functions.To evaluate the contribution of p38a...

The cathepsin K inhibitor odanacatib (ODN) is a bone formation-sparing inhibitor of osteoclastic resorption activity. This drug is currently under development for the treatment of postmenopausal osteoporosis. To support the bone safety profile of ODN, we evaluated the effects of ODN on trabecular bone hard tissue properties in the estrogen-deficient model of the ovariectomized (OVX) rhesus monkeys. Animals (n=16/group, age 11–22 years) were treated immediately af...

This study was undertaken to evaluate the effect of whole-body vibration (WBV) with extremely low-amplitude on early-stage bone healing. Experiments were conducted with an approval of the Animal Research Committee of Osaka University Graduate School of Engineering Science. Male BALB/cByJJcl mice were subjected to a 0.5 mm drill-hole surgery on a tibial diaphysis at 14 weeks of age and divided into three groups (n=8 each) from the day after the surgery, which received ...