Bone Abstracts

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...

Purpose: There is no agreed standardised method for assessing vertebral morphology. The algorithm-based qualitative technique appears to be promising in adults to rule out non-osteoporotic fractures and non-fracture deformities, however it has not been previously trialled in children. We aim to modify a technique for use with both dual energy X-ray absorptiometry and radiographs in children, and to evaluate its reliability and reproducibility.Methods: 50...

Purpose: Children have greater lifetime risks of radiation-induced complications compared to adults. In children with osteogenesis imperfecta conventional radiographs are obtained to assess spine morphometry, while DXA assesses bone density. In adults DXA is now used for both. We aim to establish whether iDXA can replace spine radiographs in the assessment of paediatric vertebral morphometry.Methods: An 18-month prospective recruitment of 200 consented c...

Osteoporosis pseudoglioma syndrome (OPPGS) is a rare autosomal recessive disorder characterised by congenital or juvenile-onset blindness, severe juvenile-onset osteoporosis, and skeletal fragility. OPPGS is caused by loss-of-function mutations in the LRP5 gene, a member of the LDL receptor family. It activates the canonical Wnt/β-catenin pathway, regulating osteoblastic bone formation. We investigated a 40-year-old Caucasian male presenting with congenital blind...

Vertebral fracture assessment (VFA) by DXA is an accepted tool in adults. However, its use in children has not been validated. The aim of this study was to validate VFA using iDXA against spinal radiographic assessment (RA) for the identification of vertebral fractures in children.Spine radiographs and VFA (L5–T2) by GE-iDXA were acquired on the same day in 80 children. Forty children were considered high-risk for fracture as their metabolic bone sp...

The oim/oim mouse resembles type III OI. Our study mimics clinical questions: is RANK-Fc as efficacious as ALN in reducing fracture number? Is RANK-Fc safe to administer to children? Is there any advantage to receiving RANK-Fc treatment post adolescence, following no treatment or ALN treatment from infancy through adolescence?All animal work was done under an IACUC-approval. Mice – n=200, WT or oim/oim. Treatment spanned a...

Objective: The purpose of this study was to determine whether osteogenesis imperfecta (OI) patients entering adulthood should continue with bisphosphonate therapy or would benefit from switching to a RANKL blockade therapy. To address this question, we used a mouse model of type III OI.Methods: Animal studies were performed under IACUC approval. OI (oim/oim) and wild-type (WT) mice were treated from 2–26 weeks with i) saline; ii) alendronat...

Background: Autocrine and paracrine Wnt signaling operates in stem cell populations and regulates mesenchymal lineage specification. In Wnt producing cells, Wntless (GPR177) supports the transport of Wnt from the trans-Golgi network (TGN) to the cell surface in vesicles, from which Wnt is then released. The retromer complex (VPS35) interacts with Wntless and retrieves Wntless from endosomes back to TGN, thereby maintaining the normal levels of Wntless protein. V-ATPases-driven...

Background: The activation of Wnt/β-catenin signaling promotes cellular senescence and induces matrix metalloproteinases (MMPs) expression in intervertebral disc (IVD). However, little is known about the effects of hyperbaric oxygen (HBO) on the Wnt/β-catenin signaling and MMPs expression in degenerated human IVDs.Materials and methods: Nucleus pulposus cells (NPCs) were separated from the degenerated disc nucleus tissue by performing sequentia...