Bone Abstracts

Background: Chronic renal, liver and heart failure in children associate with multiple skeletal complications. Increased fracture incidence often persists after transplantation and might be related to alterations in bone material properties. Moreover, it is not clear whether bisphosphonate therapy (BP) alters bone matrix mineralization in these patients.Methods: In the present study we evaluated bone mineralization density distribution (BMDD) by quantita...

Objectives: Progressive heterotopic ossification in fibrodysplasia ossificans progressiva (FOP; OMIM #135100) begins in childhood and leads to irreversible restriction of movement, functional impairment, and shortened life-span. Baseline data from an on-going, global, 3-year, natural history study (NHS) describe FOP disease characteristics, and retrospective flare-up history, causes/symptoms, and outcomes.Methods: Data from 101 subjects (recruited from 2...

Background: Previous studies with small number of boys with Duchenne Muscular Dystrophy (DMD) suggest that growth failure occurs in glucocorticoid naïve (GC) boys.Objective: To evaluate height and longitudinal growth in boys with DMD prior to GC.Method: Retrospective evaluation in boys with DMD with height measurements obtained for clinical purposes. Out of the 91 boys currently managed in Scotland, 51 had at least one height ...

Objective: FOP is a rare, severely disabling disease characterized by episodic flare-ups and accumulation of heterotopic ossification (HO) leading to restricted movement, physical disability, and early death. Data from two Phase 2 interventional studies and one natural history study (NHS) were used to evaluate whether palovarotene could reduce HO following an FOP flare-up.Methods: HO volume at the flare-up site was determined by CT at baseline and 12 wee...

Introduction: The body composition at term equivalent age of infants born preterm is different than that of infants born at term.Aim: The aim of the study was to compare the body composition in preterm and full-term infants at age 3 and 4 years.Patients and methods: Total body bone mineral content (TBBMC, g), density (TBBMD, g/cm2), and body composition (fat mass – FM, g; lean body mass – LBM, g) were measured ...

Objectives: To describe the incidence of vertebral fractures in steroid-treated children.Methods: Fractures were assessed prospectively each year for 3 years according to the Genant semi-quantitative method. Proportions of children with incident fractures were determined annually over the study period. To examine associations with baseline clinical factors, the 3-year total number of incident fractures was analyzed using multivariable Poisson regression....

Vertebral fractures (VF) defined by morphometric analysis of spine radiographs are the most common complication of osteoporosis. Those that come to medical attention, with symptoms such as back pain and kyphosis are termed clinical vertebral fractures (CVF) and account for significant morbidity and mortality. Although much progress was made in identifying loci for bone mineral density, the genetic determinants of CVF remain unclear. Here we present the initial results from a g...

Diseases affecting the musculoskeletal system are a major cost burden to society. Articular cartilage loss or damage in these diseases is detected by radiography and measuring decreases in joint space width (JSW). The early stages of the disease may remain latent and asymptomatic for many years. This forms the rationale for applying molecular marker technology (biomarkers) to identify patients prone to joint diseases. One such biomarker is cartilage oligomeric matrix protein (...

Activation of p38 MAPK by RANKL is regulated by the scaffolding protein receptor for activated C-kinase 1 (RACK1) in osteoclast precursors, whereas it is unclear whether RACK1 can also affect in mature osteoclasts. In this study, to identify that the interaction of RACK1 with c-Src is essential for osteoclast function, we generated several mutants affecting the RACK1-c-Src association. A RACK1 mutant protein (mutations of tyrosine 228 and 246 residues to phenylalanine) disrupt...

Osteoporosis is a common skeletal disorder characterized by low bone mass leading to increased bone fragility and fracture susceptibility. Little is currently known about what specific factors stimulate osteoblast differentiation from human mesenchymal stem cells (hMSCs). Therefore, the aim for this project is to determine novel factors and mechanisms involved in human bone production which can be targeted to treat osteoporosis, using gene expression profiling and bioinformati...