Bone Abstracts

Objectives: Denosumab (Dmab) is a monoclonal antibody targeting RANKL administered by sub-cutaneous injection. Given its convenient mode of administration, our goal was to assess the safety and efficacy of Dmab compared to intravenous zoledronic acid (ZA) in pediatric osteoporosis.Methods: In this one-year pilot study (NCT02632916), children 4–16 years with low-trauma fractures due to osteoporosis were randomized 1:1 to receive ZA 0.025 mg/kg or Dma...

Objectives: Denosumab (Dmab) is a monoclonal antibody targeting RANKL administered by sub-cutaneous injection. Recent reports have raised concern about the ‘rebound phenomenon’ (hypercalcemia and increases in bone turnover markers, BTM) following Dmab in adults, and during treatment in children with osteogenesis imperfecta. The purpose of this report was to explore this phenomenon in children with osteoporosis associated with lower bone turnover.<p class="abstext...

Objectives: To evaluate musculoskeletal trajectories in children with newly diagnosed Crohn’s disease (CD), and to determine whether children treated with anti-tumour necrosis factor-alpha antibody (anti-TNF, TREATED vs NAÏVE) had persistent deficits at two years.Methods: This was a single-centre prospective, observational inception cohort study. Children with CD underwent assessments within 6.5±9.5 days from diagnosis and annually for two...

Objectives: To determine the influence of conservative management upon genetically-determined metabolic derangements in osseous tissue among patients with vitamin D-dependent rickets type 2.Methods: The conservative management of the 39 patients with rickets-like diseases involved 4 stages (tab1). 1st stage included complete examination of the patient referring determination of the calcium and phosphorus in blood and urine, calcidiol and calcitriol in bl...

Neurofibromatosis type I (NF1) is a monogenetic disorder caused by mutations in the NF1 gene encoding for the Ras-GAP protein neurofibromin. Apart from benign tumour development NF1 is frequently associated with skeletal manifestations such as osteopenia or debilitating focal skeletal dysplasia. To assess a function of Nf1 in osteocytes we here apply a combinatorial approach of biophysical, histological and molecular techniques allowing differential analysis ...

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

Aim: To investigate the acute effects of oral glucocorticoids in doses used in clinical practice on biochemical indices of function of osteoclasts, osteoblasts and osteocytes.Methods: In 17 adult patients suffering from various medical pathologies requiring systemic steroid therapy that were never before treated with glucocorticoids, the glucocorticoid treatment was initiated (mean prednisolone equivalent dose of 23.1±12.7 mg/day, range 10–50 m...

Background: Association between high serum homocysteine (S-Hcy) levels and low bone mineral density (BMD) and increased fracture risk in postmenopausal women has been repeatedly documented. There are scarce data concerning S-Hcy and bone health in children and adolescents.Patients and methods: We assessed S-Hcy levels in 37 children and adolescents (22 boys and 15 girls; mean age 13.9±3.5 years) with prevalent low-energy trauma fractures (mean 3.3&#...

Objectives: To evaluate the effect of oral ibandronate on bone health in osteoporotic children and adolescents, as there are only scarce data regarding its use in pediatric population.Patients, materials, methods: We enrolled seven patients (six boys; one girl; mean age 15.3±3.8 years; range 8–18 years) with low bone mineral density (BMD) (mean 0.746±0.141 g/cm2; i.e. −3.3±1.5 S.D. z-score) and...

Background: Treatment with orally administered ibandronate is an effective way to increase bone mineral density (BMD) and reduce fracture rate in post-menopausal women and in men with osteoporosis. There are only very few reports concerning ibandronate therapy in children/adolescents, and in patients with osteogenesis imperfecta (OI), as bisphosphonates are off-label drugs in pediatrics.Case presentation: We present two patients with OI type I (14 year o...