Bone Abstracts

Objectives: To investigate whether bone health outcomes (volumetric bone mineral density, geometry and strength) is associated with adiposity and muscle in late childhood.Methods: Design: Population-based cross-sectional study nested within the Longitudinal Study of Australian Children. Participants: 11–12 year-olds attending the Child Health CheckPoint physical module. Exposures: Measures of adiposity (BMI z-score, fat ma...

Hypophosphatasia (HPP) is a rare genetic disease characterised by low tissue-nonspecific alkaline phosphatase activity, causing defective mineralisation of bone and teeth. There is limited data on the measurement of bone mineral density (BMD) and body composition in these children.Objectives: To assess whether BMD and lean body mass (LBM) in treatment naïve children with HPP correlate with functional outcomes using the 6-minute walk test (6MWT)....

Introduction: Intracellular fat within muscle and visceral tissue has been suggested to adversely influence bone development.Design: We aimed to compare associations between intramuscular and subcutaneous fat and cortical bone outcomes in young adults, in cross-sectional analyses based on the Avon Longitudinal Study of Parents and Children.Method: Data were collected from a research clinic conducted at 17 years of age. Intramuscula...

see PP383....

Introduction: Osteoporosis and fragility fractures are recognized complications of rheumatoid arthritis (RA). Previously Riches et al. described a patient with celiac disease and severe osteoporosis in whom neutralizing antibodies to osteoprotegerin (OPG) were present. The aim of this study was to determine if OPG autoantibodies were present in patients with RA and other rheumatic diseases and to relate these to clinical features.Methods: We dev...

Strontium ranelate is a promising drug used in the treatment of osteoporosis. This drug has a unique dual effect on bone turnover, simultaneously increasing bone formation by osteoblasts (anabolic effect) and decreasing bone resorption by osteoclasts (antiresorptive effect). The goal of this study was to evaluate: i) anabolic effects of strontium ranelate on the formation of bone-like mineralized matrix in osteoblast cell cultures and ii) changes the drug could cause on the ma...

Metatropic dysplasia (MD) was first described by Maroteaux et al. in 1966. Its name was derived from the Greek word metatropos which means ‘changing pattern’ because individuals with this diagnosis begin life with a short-limbed dysplasia and a long trunk with narrow chest, and over time their dysplasia becomes short-trunked due to progressive kyphoscoliosis1. It is now recognized that MD is caused by gain-of-function mutations in transie...

Objective: Hypophosphataemic rickets (HPR) is associated with high concentrations of fibroblast growth factor 23 (FGF23). Chronically elevated FGF23 may impact on cardiac function and the skeleton. There is little evidence on how FGF23 changes with time in HPR or how it is affected by treatment. The aim of this study was to evaluate changes in FGF23 concentrations over time in patients with HPR.Methods: Retrospective data was collected from our Metabolic...

Fibroblast growth factor-23 (FGF23) is a major regulator of phosphate metabolism often elevated in genetic hypophosphataemic disorders and in chronic kidney disease–bone mineral disorder (CKD–BMD). Recent studies have identified relationships between FGF23 and vitamin D.Objectives: To determine the relationship between vitamin D and FGF23 metabolism in CKD.Method: We used randomized samples from patient...

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 ...