Bone Abstracts

Objectives: The ability to discriminate bone fractures that result from non-accidental injuries and those that result from underlying bone fragility is limited by the lack of clinical instrumentation to directly measure bone mechanical properties. The BioDent Hfc (Active Life Scientific) is an experimental device that has been validated for measuring cortical bone fracture resistance in adults. We are currently developing protocols to test the feasibility of its use in infants...

Background and aim: Eight cases of bisphosphonate-associated osteonecrosis of the external auditory canal have been reported in case-reports. Our aim was to describe the incidence of external auditory canal and middle ear diseases in Danish patients exposed to bisphosphonates in the treatment of osteoporosis.Methods: The study was a retrospective, nationwide cohort study, within the Danish population of approximately 5.6 million individuals and based on ...

Genetic skeletal diseases (GSDs) are an extremely diverse and complex group of rare genetic diseases that primarily affect the development and homeostasis of the osseous skeleton. There are more than 450 unique and well-characterised phenotypes that range in severity from relatively mild to severe and lethal forms. Although individually rare, as a group of related genetic diseases, GSDs have an overall prevalence of at least 1/4000 child. Qualitative defects in cartilage struc...

Biographical DetailsMichael D Briggs obtained his PhD at the MRC Clinical Research Centre, Harrow, studying the genetic basis of osteogenesis imperfecta. He undertook postdoctoral work at UCLA identifying the genetic basis of chondrodysplasias. In 1996 Mike moved to Manchester as an AR-UK Fellow to continue studying disease mechanisms in chondrodysplasia. In 2004 he was awarded a Wellcome...

The healing of bone fractures is a tightly regulated process where released growth factors and cytokines interplay within an inflammatory environment in order to reestablish the functional bone. Recent studies have suggested that endothelial cells may dedifferentiate into mesenchymal multipotent cells via a mechanism called endothelial-to-mesenchymal-transition (EndoMT). Transforming growth factor-β (TGF-β) plays a critical role inducing EndoMT. Subsequent differenti...

Metabolic bone disease (MBD) of prematurity is a well-recognized complication of preterm birth. Yet there is limited evidence for the optimal assessment, monitoring, and subsequent bone health management.Retrospective audit of 171 infants born <32 weeks’ gestation between November 2012 and January 2014 at three Monash Health neonatal units (Melbourne, Australia) was undertaken. Infants had mean gestational age (GA) 28.6±2.1 weeks and birth ...

Screening gene function in vivo is a powerful approach to discover novel drug targets in the human genome (Nat Rev Drug Discov 2 38–51, 2003). We present data for 3776 distinct gene knockout (KO) mouse lines with viable adult homozygous mice generated using both gene-trapping and homologous recombination technologies. Bone mass was determined from PIXImus DEXA scans of male and female mice at 14 weeks of age and by microCT analyses of bones from ...

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Background: Juvenile Paget’s disease (JPD) is a rare autosomal-recessive condition. The disease is typically diagnosed in infants or young children and characterized by a generalized increased in bone turnover, bone pain, skeletal deformity and increased risk of pathological fractures. In our knowledge, inactivating mutations in the TNFRSF11B gene, which encodes osteoprotegerin, cause JPD, yet. There are no randomized controlled trials which to offer the optimal form of t...

Mineralized tissues like bone, articular calcified cartilage or mineralized turkey leg tendon (MTLT) are build by a composite of hydroxyapatite nano-particles and organic matrix. In bone and MTLT the matrix is formed by collagen type-I, but in contrast to bone in MTLT the collagen is uniaxial orientated, while in cartilage the matrix consists of collagen type-II and proteoglycans.Composition/orientation differences were investigated by a new scanning aco...