Bone Abstracts

Low bone mineral density (BMD) is an important and modifiable risk factor for fracture in postmenopausal women with osteoporosis. Denosumab (DMAb) shows a stronger relationship between BMD increases and antifracture efficacy than oral bisphosphonate (BP) therapies. Subjects who remain at higher risk of fracture despite current BP therapy need treatment. In two studies, DMAb significantly increased BMD and decreased bone turnover markers vs a BP (ibandronate (IBN) or risedronat...

Background: Oral glucocorticoids (GC; prednisolone dose of 0.75 mg/kg per day), help to preserve muscle strength and prolong independent walking in boys with DMD. This study compared longitudinal growth, body mass index (BMI), bone mineral density (BMD), vertebral fractures (VFs) and ambulatory status in boys with DMD on daily (DAILY) or intermittent (INTER; 10 days on & 10 days off), oral GC regimens.Methods: 50 DMD boys from two UK centres were inc...

Proteoglycans is a component of extracellular matrix, forming a mesh around structural proteins including collagen and elastin, and providing elasticity to tissue structure. Proteoglycans is formed by the attachment of glycosaminoglycans to a core protein, a process that requires three enzymes: galactosyltransferase-1, galactosyltransferase-2 and glucoronosyltransferase-1, encoded by B4GALT7, B3GALT6 and B3GAT3 respectively. B3GALT6 mutation...

Introduction and objective: Adolescent idiopathic scoliosis (AIS) is a three-dimensional spinal deformity with lateral curvature of ≧10 degree measured by Cobb angle. Previous studies reported that ~30% of female AIS patients had systemic osteopenia which could persist beyond skeletal maturity and that was one of the important prognostic factors. The aim of this study is to investigate the incremental prognostic value of osteopenia on curve progression in AIS as defined...

Introduction: Children with chronic kidney disease (CKD) who suffer from growth retardation have usually normal circulating GH but low IGF1, suggesting GH resistance. We have previously shown in growth retarded CKD rats an increase in suppressor of cytokine signaling 2 (SOCS2) levels, a key negative regulator of GH signaling. SOCS2 spontaneously mutated (HG) mice show an exaggerated body growth and increased bone mass. We investigated growth and GH receptor (GHR) signaling in ...

Background: Mucopolysaccharidosis IV is a rare, autosomal recessive disorder. Common presenting features include severe short stature, kyphoscoliosis, genu valgum, cloudy cornea, osteoporosis, mild coarsening of facial features, joint restriction in some large joints. Skeletal dysplasias should be considered in its differential diagnosis. Here we present a case whose initial diagnosis was Mucopolysaccharidosis IV and was diagnosed with osteogenesis imperfecta (OI) by COL1A2 mu...

Background: Children with special needs may be greater risk of vitamin D deficiency due to decreased mobility and outdoor play, concomitant medications that increase catabolism of vitamin D, reduced nutritional intake and low body weight. Gastrostomy-fed children receiving a nutritionally complete formula may still be at risk of vitamin D deficiency due to the above factors.Objective: The objective of this study is to assess the vitamin D status of speci...

Background: A 3 month-old boy was born at term, to non-consanguineous parents by spontaneous vaginal delivery, in good condition, weighing 4.19 kg. Newborn examination, including eyes, was normal prior to discharge. He has two older brothers in good health.Presenting problem: He was admitted at 7 days of life with focal seizures and hypocalcaemia (1.5 mmol/l), hypomagnesaemia (0.6 mmol/l), hyperphosphataemia (3.7 mmol/l) and inappropriately low parathyro...

The relationship between BMD T-score and FX risk has not been established in patients receiving osteoporosis therapy. In the FREEDOM Extension study, continuous DMAb therapy for up to 10 years increased BMD levels with no therapeutic plateau at lumbar spine or TH [Bone et al, ASBMR 2015]. Such improvements would only be meaningful if associated with FX reductions. We investigated the relationship between TH BMD T-score and NVFX in women who received DMAb during FREEDOM and tho...

Odanacatib (ODN), a selective oral inhibitor of cathepsin K, is in development for the treatment of osteoporosis. In the Phase 3, Long-Term ODN Fracture Trial (LOFT; NCT00529373), ODN significantly reduced fracture risk. This imaging sub-study primarily investigated the effect of ODN on volumetric BMD (vBMD) of the lumbar spine (LS) and total hip (TH) using quantitative computed tomography (QCT).Women aged ≥65 without baseline radiographic vertebra...