Searchable abstracts of presentations at key conferences on calcified tissues

ba0002p166 | (1) | ICCBH2013

Severe metabolic bone disease of prematurity following continuous veno–veno haemofiltration

Elder Charlotte , Arundel Paul , Perring Jeff , Bishop Nick

A first twin born at 28 weeks gestation weighing 630 g underwent an end-to-end anastamosis for colonic stricture on day 92 of life. He collapsed with severe Escherichia coli sepsis post-operatively and became anuric. Veno–veno haemofiltration (CVVH) was instituted as a life-preserving measure, continuing for 3 days.On day 119, osteopaenia and rachitic changes were noted on a chest X-ray. Review of his prior biochemistry showed a precipitous...

ba0003pp16 | Bone biomechanics and quality | ECTS2014

Inhibition of RANKL-mediated bone remodeling decreases bone damage and improves strength in response to fatigue loading

Bonnet Nicolas , Gerbaix Maude , Kostenuik Paul , Ominsky Mike , Ferrari Serge

Antiresorptives consistently improve bone mass and structural strength in normally- and under-loaded bones, but concerns have been raised regarding potential effects on skeletal adaptation to fatigue loading, including damage accumulation and atypical fractures. We thus inhibited or activated osteoclasts with OPG-Fc or RANKL treatment, respectively, and evaluated bone damage and strength after fatigue in the early and later phases of repair. Adult male mice were treated with R...

ba0003pp19 | Bone biomechanics and quality | ECTS2014

Scanning acoustic microscopy reveals heterogeneity of mechanical properties due to collagen orientation in mice cortical bone

Blouin Stephane , Puchegger Stephan , Klaushofer Klaus , Roschger Paul , Fratzl Peter

The local mechanical properties of bone are influenced not only by the material chemical composition but also by the spatial arrangement of the component’s e.g. orientation of collagen matrix. However, not much is known about local elastic modulus variations in cortical bone. Our goal was to use acoustic imaging to map elastic properties of murine bone with a several microns resolution. Rodent long bones exhibit a permanent growth with endosteal/periosteal bone formation ...

ba0003pp22 | Bone biomechanics and quality | ECTS2014

Bone matrix mineralization is preserved during perimenopausal stage in healthy women

Misof Barbara , Roschger Paul , Recker Robert , Klaushofer Klaus

Menopause is accompanied by a decrease in bone mineral density which can be caused by a reduction in bone volume and/or degree of bone matrix mineralization. Both of them are suggested to contribute to the increased fracture risk in postmenopausal individuals. In the present work, we aimed for information whether a drop in bone matrix mineralization is occurring in the perimenopausal stage of women. For this purpose, we measured the bone mineralization density distribution (BM...

ba0003pp37 | Bone biomechanics and quality | ECTS2014

Bone matrix mineralization after sclerostin antibody treatment in a mouse model of osteogenesis imperfecta

Roschger Andreas , Roschger Paul , Kneissel Michaela , Rauch Frank

Children with osteogenesis imperfecta (OI) still suffer from frequent fractures, despite bisphosphonate treatment. Thus new therapeutic approaches are needed. Sclerostin is a protein that is thought to inhibit bone formation. Treatment with sclerostin antibodies (SclAB) increases bone mass in animal models and in clinical trials and may be a rational therapy for OI as well.Transgenic (TgOI) Col1a1Jrt/+ mice were gene...

ba0004oc4 | (1) | ICCBH2015

Increased bone matrix mineralization in treatment-naive children with Crohn's disease

Roschger Paul , Misof Barbara , Klaushofer Klaus , Rauch Frank , Ward Leanne

Background: Crohn’s disease (CD) is an inflammatory bowel disease which affects many organ systems including the skeleton. In children with CD, bone mineral density is frequently low. Bone metabolic abnormalities, including lower biochemical measures of bone turnover (NTX) as well as decreased bone formation indices at the tissue level have been reported. The aim of our study was to gain information on the bone matrix mineralization in CD.Methods: W...

ba0004p88 | (1) | ICCBH2015

Vitamin D and bone health: A practical clinical guideline for management in children and young people in the UK

Thurston Anne , Bowring Claire , Shaw Nick , Arundel Paul

Background: There is currently considerable clinical and academic interest in vitamin D in children and young people. This partly relates to recognition of a resurgence of symptomatic vitamin D deficiency with reports of children presenting with rickets or hypocalcaemic symptoms. An additional development has been the recognition that vitamin D may have a physiological extraskeletal role beyond its traditional function as a key regulator of calcium and bone metabolism.<p c...

ba0004p112 | (1) | ICCBH2015

Type V osteogenesis imperfecta: confirmation of highly characteristic radiographic findings in early infancy

Arundel Paul , Offiah Amaka , Bishop Nick , Ehtisham Sarah

Type V OI is characterised by interosseous membrane calcification and hyperplastic callus formation, but the infantile phenotype is less well recognised. In 2012 Arundel et al. described distinctive radiographic changes in an infant with type V OI. We report two further male infants (with genetic confirmation of type V OI) confirming the highly characteristic and consistent radiographic appearances that should aid early diagnosis.Case 1 – P...

ba0004p118 | (1) | ICCBH2015

Changes in total body and regional bone mass in relation to body composition in children with osteogenesis imperfecta treated with pamidronate

Sritharan Shironisha , Gopal-Kothandapani Jaya Sujatha , Bishop Nick , Dimitri Paul

Background: In patients with osteogenesis imperfecta (OI), a rise in age-specific total body and regional bone mass is well recognised. In contrast changes in body size-adjusted bone mass in relation to changes in body composition following pamidronate therapy remain relatively unexplored.Methods: Changes in total and regional bone mass in relation to body composition in children with OI receiving pamidronate (3 mg/kg per day over 3 days, 3 monthly) were...