Bone Abstracts

Background: Osteoporosis is common in subjects with Duchenne muscular dystrophy (DMD). Studies in paediatric DMD identified a high frequency of fragility fractures but there are no studies in the adult population. Recent updated international standards of care (2018) for children and adults with DMD recommend the following for bone monitoring:- Lateral thoracolumbar spine x-rays to screen for vertebral fracture (1–2 yearly if on glucocorticoid; 2&#1...

Prof. David Little received his Medical Degree from the University of Sydney where he is Conjoint Professor of Paediatrics and Child Health, specialising in Orthopaedic Surgery. Prof. D Little is Head of Orthopaedic Research and Biotechnology at The Children’s Hospital at Westmead, part of the Sydney Children’s Hospital Network. He has broad clinical interests in Children’s Orthopaedi...

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Tibial dysplasia, which leads to fracture and pseudarthrosis, occurs in around 4% of children with NF1, and also in children with no underlying disorder. Pseudarthrosis of the fibular may or may not be present, or as an isolated entity, as can pseudarthrosis in the forearm (rare). Other bone problems faced by individuals with NF1 are scoliosis (20%), pectus excavatum/carinatum (12%), and sphenoid wing dysplasia (7%). Dural ectasia and plexiform neurofibromas can also affect th...

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Background: Children with inflammatory bowel disease (IBD) have reduced bone mineral density (BMD). The aim of this study was to investigate whether changes in patient’s cellular immune response correlate with reductions in BMD.Method: Children undergoing lower gastrointestinal endoscopy disease were approached with an aim of recruiting 15 patients newly diagnosed with Crohn’s Disease (CD) and 15 healthy controls. Lymphocytes were isolated from...

This paper reviews clinical reports of vitamin D deficiency rickets in neonates from 1930 onwards. In 24 reports there was good evidence of maternal deficiency. In some the diagnosis of the rickets led to the identification of symptomatic osteomalacia in the mothers; several had been severe and longstanding. Of the 15 mothers who had assays for serum 25-hydroxyvitamin D (25OHD) 13 had values <25 nmol/l (10 ng/ml) and 10 had values <12.5 nmol/l (5 ng/ml).<p class="a...

Crohn’s disease often results in abnormalities in bone strength, and ultimately increases the risk of fragility fracture. Up to 55% of patients with Crohn’s disease have bone mineral density in the osteopenia range up to 50% of osteoporosis. Glucocorticoid is frequently used in the treatment of Crohn’s disease and is associated with osteoporosis and increased fracture risk. It has been reported that osteoporotic fractures in patients with Crohn’s disease ar...

Objectives: Young male jockeys undertake calorie restriction and high volumes of physical activity during periods of musculoskeletal growth and development. Previous research shows that jockeys have compromised bone health1–4 and display disordered eating5–7. Restricted intakes of calcium and vitamin D, together with excessive amounts of exercise, increase the risk of osteoporosis in males8. The aim of this study was to establish whet...

Osteoclasts are highly specialized cell types, responsible for the resorption of bone matrix. Coordinated with osteoblasts they contribute to a proper bone turnover. An impaired or reduced function of the osteoclast leads to a pathogenic increase of the bone mass and finally osteopetrosis. Lysosomal hydrolases, as exemplied by CTSK and Acp5 (TRAP), are known to play an important role in the function of osteoclasts, and knockout mouse models of these proteins develop a bone res...