Bone Abstracts

Objectives: Neurofibromatosis 1 (NF1) is one of the most common autosomal dominant diseases. Skeletal involvement such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Moreover, there is a growing evidence that reduced bone mineral density (BMD), is a common feature of NF1 subjects. The aim of the study was to evaluate the usefulness of Bone Mineral Density and Quantitative Ultrasound (QUS) at phalanxes in the assessm...

Objectives: To assess lumbar spine (LS) BMD Z-scores and the prevalence of vertebral fractures using DXA lateral vertebral (VFA) assessment in children and adolescents with chronic illnesses on GCs.Methods: All children between the ages of 5 and 17 years with chronic non-malignant illnesses who were on GCs (intravenous or oral) for greater than 3 months duration were evaluated. Study participants were children attending the paediatric sub-specia...

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 the clinical outcomes of intravenous bisphosphonate treatment in children with mild-moderate osteogenesis imperfecta (OI) who had progressed from active bisphosphonate treatment to maintenance therapy for >2 years.Methods: A retrospective review was conducted on 17 patients with mild-moderate OI. Clinical data, fracture history, biochemistry, dual energy X-ray absorptiometry (DXA) parameters, vertebral measurements, bone age a...

Sclerosing bone dysplasias (too much bone diseases) are divided into two major groups, one group caused by impaired bone resorption and the other by increased bone formation. The former comprises a group of osteopetrosis (disorders due to abnormal osteoclastogenesis or osteoclast dysfunction) with relatively homogeneous skeletal phenotypes. By contrast, the latter encompasses a complex group of disorders with heterogeneous pathogeneses and diverse skeletal phenotypes.<p cl...

Objective: Expansion of bone marrow, accumulation of iron, growth failure and delayed puberty affect bone health in thalassemia; data on bone density and geometry are scarce. Our objective was to assess bone density (by Dual Energy X-ray Absorptiometry, DXA) and geometry (by peripheral quantitative computed tomography, pQCT) in children with thalassemia major.Methods: Children with thalassemia were recruited from a hematology clinic in Pune (India) (Mean...

Objectives: Losartan is an angiotensin II receptor type 1 (AT1) antagonist. Losartan reduces circulating TGFb concentrations in a variety of myopathic models. We hypothesised that losartan administration to the murine osteogenesis imperfecta model OIM would result in lower circulating TGFb and CTX (bone resorption marker) and increase bone mass.Methods: All procedures were approved by UConn Health Institutional Animal Care and Use Committee and performed...

Introduction: Treatment in thyroid function disorders, which lead to clinical hypothyroidism, is replacement with levothyroxine. We aimed to investigate the effect of long-term levothyroxine therapy on vitamin D metabolism and bone mineral density in children.Materials and methods: Twenty-seven children with hypothyroidism receiving levothyroxine therapy (M/F: 13/14, mean age, 12.1±0.7 years) and 21 healthy controls (M/F: 13/8, mean age, 11.8±0...

Objectives: To examine the bone mineral density (BMD) response to i.v. pamidronate (Group 1) vs i.v. zoledronic acid (Group 2) in non-ambulatory children and young adults with severe cerebral palsy or other neuromuscular disorders.Methods: A total of 50 non-ambulatory children and young adults, (mean age 11.3 years, range 2.1–32) with low BMD and/or history of fractures were retrospectively studied. Thirty-nine patients (30 spastic quadriplegic cere...

Background: Among body composition phenotypes there is no consensus on definition and consequences of sarcopenic obese phenotype. The recommended method to quantify the muscle mass is the dual energy X-ray absorptiometry (DXA), based on measurement of total fat mass and appendicular lean mass. A high BMI may provide a great bone mineral density (BMD) in many individuals, but it does not guarantee optimal bone strength. The aim of our study is to investigate the influence of sa...