Bone Abstracts

It is well established that tumor expression of Gli2, a Hedgehog family transcription factor, contributes to tumor-induced bone disease. Our previous studies investigating genetic inhibition of Gli2 expression in tumor cells have shown promise for the development of therapeutics. While many groups have focused on developing upstream Hedgehog inhibitors for soft-tissue tumors that aberrantly express Gli proteins, we have previously demonstrated that the expression of Gli2 in tu...

Objective: Children with hypophosphatasia (HPP) treated with asfotase alfa in a Phase 2 study (NCT00952484) and its open-label extension (NCT01203826) experienced significant improvements in skeletal mineralization and physical function that were sustained through 5 years of treatment (1). Herein, we report data from these studies with a maximum of 7 years of treatment.Methods: Children with HPP aged 6–12 years at baseline received asfotase alfa (3 ...

Objective: To evaluate safety and efficacy after 5 years of treatment with asfotase alfa in adolescents and adults with hypophosphatasia (HPP) in a Phase 2, open-label, randomized, dose-ranging study (NCT01163149).Methods: Treatment with subcutaneous asfotase alfa 0.3 or 0.5 mg/kg per d was compared with no treatment (control) for 6 months in patients aged 13–66 years. After 6 months, all patients (treatment and control groups) received active treat...

Objectives: To evaluate musculoskeletal trajectories in children with newly diagnosed Crohn’s disease (CD), and to determine whether children treated with anti-tumour necrosis factor-alpha antibody (anti-TNF, TREATED vs NAÏVE) had persistent deficits at two years.Methods: This was a single-centre prospective, observational inception cohort study. Children with CD underwent assessments within 6.5±9.5 days from diagnosis and annually for two...

Introduction: Vitamin D is known to be important for bone health. Still, the significance of serum 25-hydroxy vitamin D concentrations (s-25OHD) for hip fracture risk of the elderly is uncertain. Discordant findings may in part be explained by difficulties of RCTs or large cohort studies to reach both the frail and the healthy elderly. The objectives of this study were to determine the risk of hip fractures of the elderly related to s-25OHD, including both the frail and the he...

Objective: Total body less head (TBLH) and spine are the recommended DXA sites for bone health assessment in children and adolescents. However, inter-machine differences will affect use and interpretation of results in clinical care and research applications. We examined BMC and areal-BMD (aBMD) at 4 skeletal sites among healthy children to identify the magnitude of inter-machine differences in Z-scores.Methods: BMDCS evaluated healthy participa...

Background and Objectives: The relations among cortical volumetric bone mineral density (CortBMD) and comprehensive measures of mineral metabolism have not been addressed in chronic kidney disease (CKD). The aim was to identify determinants of CortBMD in childhood CKD. A secondary objective was to assess if CortBMD was associated with subsequent fracture.Design/participants: This prospective cohort study in children, adolescents and young adults (ages 5&...

Objectives: Special dual-energy X-ray absorptiometry (DXA) protocols permit quantification of bone mineralization, fat mass, and fat distribution in infants. Our objective was to evaluate the accuracy and precision of a multiscan acquisition protocol designed to allow for reflection and imputation analysis for regions with movement.Methods: The IMPAACT P1084s Study assesses bone and kidney safety of antiretrovirals used for PMTCT. Newborns received a spi...

see PP385....

Introduction: Fracture risk assessment using FRAX® estimates 10-year fracture risk (FR10) at major sites (Maj_FR10) and hip (Hip_FR10). In 2012, in Newcastle, UK, a strategy was agreed to share data between General Practice (GP), Clinical Commissioning Groups and local hospitals to identify patients at high FR10.Methods/design: FRAX® and RAID® (a health reporting, analysis and intelligence delive...