Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p73 | Bone development/growth and fracture repair | ECTS2016

Combination of novel two-photon photopolymerised scaffolds and bioactive elastin-like-recombinamers induce bone regeneration

Kampleitner Carina , Hildebrand Gerhard , Liefeith Klaus , Gonzalez Constancio , Rodriguez-Cabello Jose Carlos , Markus Andrea , Dullin Christian , Alves Frauke , Hoffmann Oskar

Non-healing fractures caused by trauma, disease or tumour resection demand the use of bone grafts to support and stimulate the healing process. We sought to develop and evaluate the effect of a novel bioactive biodegradable biomaterial designed to fill large bone lesions and to improve bone healing. We used two-photon polymerised synthetic polymer scaffolds composed of lactide (LA), caprolactone (CL), and methacrylate (MA) with varying LA: CL ratio and percentage of methacryla...

ba0004is19biog | (1) (1) | ICCBH2015

Rare sclerosing bone dysplasias

Nishimura Gen

Biographical DetailsDr Gen Nishimura is Radiologist-in-Chief, Department of Pediatric Imaging, Tokyo Meteropolitan Children’s Medical Center, Tokyo, Japan. After obtaining the medical degree at Keio University School of Medicine in Tokyo, Dr G Nishimura started his career of diagnostic radiology in the late 1970s. During the exposure to pediatric radiology in early 1980s, he develope...

ba0001pp451 | Osteoporosis: treatment | ECTS2013

Estimation of vertebral and femoral strength during the first three years of denosumab therapy using an alternative smooth non-linear finite element methodology

Zysset Philippe , Pahr Dieter , Engelke Klaus , Genant Harry , McClung Michael , Kendler David , Recknor Christopher , Kinzl Michael , Schwiedrzik Jakob , Museyko Oleg , Wang Andrea , Libanati Cesar

Denosumab subcutaneous administration every 6 months reduced the incidence of new fractures in postmenopausal women with osteoporosis by 68% at the spine and 40% at the hip over 36 months compared with placebo in the FREEDOM study (Cummings et al., NEJM, 2009:361:756). This efficacy was supported by differential improvements from baseline in vertebral and femoral strength at 36 months (18.2 and 8.6%, respectively) estimated by an established voxel-based finit...

ba0001pp474 | Other diseases of bone and mineral metabolism | ECTS2013

Insertion of the clcn7 gene mutation pG213R in mouse induces autosomal dominant osteopetrosis type II

Fattore Andrea Del , Gray Amie K , Ichikawa Shoji , Chu Kang , Mohammad Khalid S , Capannolo Marta , Muraca Maurizio , Teti Anna , Econs Michael J , Alam Imranul

Autosomal dominant osteopetrosis type II (ADO2) is a rare osteosclerotic disease due heterozygous missense mutations of the CLC7 gene encoding the type seven chloride channel. Our two labs independently generated the first C57 black 6 (B6) mouse model of ADO2 by inserting the pG213R-clc7 mutation. Homozygous mice showed lack of tooth eruption and died within 30 days of age with severe osteopetrosis and central nervous system degenera...

ba0002oc8 | Biology | ICCBH2013

Generation of the first mouse model of autosomal dominant type II osteopetrosis harbouring the pG213R-clc7 mutation

Del Fattore Andrea , Gray Amie , Ichikawa Shoji , Chu Kang , Mohammad Khalid S , Capannolo Marta , Capulli Mattia , Muraca Maurizio , Econs Michael J , Teti Anna , Alam Imranul

Autosomal dominant type II osteopetrosis (ADO2) is a rare osteosclerotic disorder due to heterozygous missense mutations of CLC7 gene encoding the type 7 chloride channel. Our two labs (L’Aquila and Indianapolis) independently generated the first C57 black 6 (B6) mouse model of ADO2 by inserting the pG213R-clc7 mutation. We created pG213R-clc7 KI mice using a gene targeting approach. Homozygous mice showed lack of tooth eruption and died within ...

ba0003pp354 | Osteoporosis: treatment | ECTS2014

Denosumab treatment in women with osteoporosis reduces hip cortical porosity

Zebaze Roger M , Libanati Cesar , McClung Michael R , Zanchetta Jose R , Kendler David L , Hoiseth Arne , Wang Andrea , Ghasem-Zadeh Ali , Seeman Ego

Bone strength is influenced by cortical thickness, area, mass and porosity, all of which contribute to nonvertebral fracture risk. Cortical porosity is one parameter of structural decay associated with bone fragility. This is caused by unbalanced and accelerated remodelling of Haversian units which enlarge, coalesce and fragment the cortex. Antiresorptive therapies will limit progression of cortical porosity; reducing existing porosity would be a goal for those already at incr...

ba0005oc3.2 | Clinical trials, FGF-23 and focal osteoporosis | ECTS2016

Effects of Denosumab (Dmab) on bone matrix mineralization: results from the phase 3 FREEDOM trial

Dempster David W , Brown Jacques P , Yue Susan , Farlay Delphine , Rizzo Sebastien , Song Jenny , Wang Andrea , Wagman Rachel B , Boivin Georges

Low fracture (FX) incidence has been demonstrated in women with postmenopausal osteoporosis (PMO) treated with DMAb for up to 10 years in the FREEDOM extension [Bone ASBMR 2015]. Bone biopsy-based assessment of DMAb’s effects at the tissue level has demonstrated a low remodelling rate consistent with DMAb’s mechanism of action (Reid JBMR 2010; Brown JBMR 2014). From FREEDOM, we report the effects of DMAb on bone matrix mineralization in women who un...

ba0005p244 | Genetics and Epigenetics | ECTS2016

NBAS is the gene mutated in two patients affected by Acrofrontofacionasal Dysostosis type 1

Palagano Eleonora , Zuccarini Giulia , Mantero Stefano , Conte Daniele , Angius Andrea , Uva Paolo , Prontera Paolo , Vezzoni Paolo , Villa Anna , Merlo Giorgio , Sobacchi Cristina

Acrofrontofacionasal Dysostosis type 1 (AFFND1) is a rare human syndrome (estimated prevalence lower than 1:1,000,000), characterized by bone abnormalities in addition to other multiple congenital anomalies and intellectual disability. Only four AFFND1 families, three of Brazilian and one of Indian origin, have been described so far and an autosomal recessive pattern of inheritance has been suggested. The patients are severely affected: the main clinical features are intellect...