Bone Abstracts

Objective: To evaluate bone and muscle mass in children with inflammatory bowel disease (IBD) following infliximab (IFX) therapy.Methods: Prospective longitudinal study of 19 children (12 males), 17 crohn’s disease (CD), 1 ulcerative colitis (UC), 1 IBD unclassified (IBDU) of bone evaluation commencing treatment with IFX. Bone and muscle parameters were measured by pQCT at the non-dominant distal radius at 4 and 66% at baseline and 6 months. pQCT pa...

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved medical treatment for pediatric-onset hypophosphatasia (HPP), which is caused by deficient tissue-nonspecific alkaline phosphatase activity. We detail the long-term efficacy of AA observed from the pediatric clinical trial program.Methods: Efficacy data collected to study completion were pooled from 3 open-label, multicenter investigations of children who manifested HPP s...

Participation in high impact sports during growth increases bone quality. Gymnasts have previously displayed increased bone mass and strength at both the upper and lower limbs compared with controls. However, it is not yet understood how bone microarchitecture is affected by gymnastics participation, and if this differs based on gymnastics discipline. Therefore, the objective of this study was to investigate the influence of gymnastics discipline on bone microarchitecture in a...

Introduction: Paget’s disease of bone (PDB) has an autosomal-dominant mode of inheritance in one-third of cases. The germinal SQSTM1/P392L mutation is the most frequent mutation, present in 40% of patients with a familial form of PDB, and 8% of unrelated patients. Fibrous dysplasia (FD) is a rare bone disorder, mono or polyostotic, caused by post-zygotic mutations in GNAS gene, for which a PCR-clamping method was developed to ease their detection and avo...

Objectives: To evaluate musculoskeletal development using pQCT and DXA in childhood onset inflammatory bowel disease (IBD).Methods: Prospective longitudinal study with 12 months follow-up in 43 children (23 males) with IBD: 30 crohn’s disease(CD), 13 ulcerative colitis (UC) and inflammatory bowel disease unclassified (IBDU). pQCT at 4% and 66% radius, DXA for total body (TB), lumbar spine (LS) bone mineral content were assessed at baseline and 12 mo...

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...

Poor bone mineralization remains a major health problem in patients with Thal and has been linked to functional zinc deficiency despite adequate dietary intake. The global etiology of poor bone mineralization includes inadequate dietary intake of calcium and vitamin D, endocrinopathies leading to disturbed calcium homeostasis, dysregulation of the GH–IGF1 axis, and delayed puberty, all resulting in limited growth, decreased bone formation and increased bone resorption. Th...

Clinical trials involving drug therapies for postmenopausal osteoporosis typically compare effects of the active drug combined with vitamin D (vit D) and calcium (Ca) vs vit D and Ca supplementation on its own. Bone strength is estimated based on the amount of bone, frequently expressed as bone mineral density determined by dual X-ray absorptiometry, and quality of bone, hardly measured in clinical practice.The purpose of the present study was to compare...

The neuro-hypophiseal hormone oxytocin (OT) is a novel anabolic regulator of bone mass (Tamma et al. PNAS, 2009), upregulating expression of critical osteoblast transcription factors. These effects are mediated by oxytocin receptor, a GPCR expressed by osteoblasts. Recently an increasing number of reports indicates that GPCRs could be targeted to the nuclear membrane; prostaglandin receptors, endothelin receptors and β-adrenergic receptors among others (...

Introduction: Currently, the only available method to reliably predict osteoporotic fractures is represented by bone mineral density (BMD) measurements on proximal femur or spine, which require the use of X-rays. Aim of this study is to illustrate working principles and feasibility of a new ultrasound (US) method for bone densitometry and osteoporosis diagnosis applicable on both proximal femur and spine.Methods: A new fully automatic algorithm was devel...