Searchable abstracts of presentations at key conferences on calcified tissues

ba0003pp185 | Chondrocytes and cartilage | ECTS2014

Disturbed cartilages of the mandible in achondroplasia are associated with defective mandible shape and position

Duplan Martin Biosse , Di Rocco Federico , Heuze Yann , Gaudas Emilie , Komla-Ebri Davide , Kaci Nabil , Benoist-Lasselin Catherine , Legeai-Mallet Laurence

FGFR3 activating mutations are responsible for achondroplasia (ACH), the most common form of dwarfism. ACH clinical features include short stature, midface hypoplasia, frontal bossing and prognathism and both endochondral and membranous ossifications are disturbed. It is unknown if abnormal mandibles are present in ACH. To date, it is believed that primary (Meckel’s) and secondary (angular and condylar) cartilages play important roles in determining the final shape and po...

ba0003pp372 | Other diseases of bone and mineral metabolism | ECTS2014

Comparative effect of Denosumab vs Teriparatide on bone and energy metabolism

Rubio Veronica Avila , Rodriguez Cristina Novo , Fontana Beatriz Garcia , Martin Antonia Garcia , Garcia Rebeca Reyes , Santana Sonia Morales , Torres Manuel Munoz

Recent studies have demonstrated the role of osteocalcin in energy metabolism regulation having a connection between this and bone metabolism. According to this, anti-osteoporotic drugs may exert different effects on energy metabolism. Thereby, our aim is to evaluate the effects of antiresorptive (Denosumab) and osteoanabolic (Teriparatide) drugs that reduce or increase respectively osteocalcin levels, on energy and bone metabolism by assessing of undercarboxilated osteocalcin...

ba0005p76 | Bone development/growth and fracture repair | ECTS2016

Multicentre study reveals poor correlation between in vitro and in vivo assessments of biomaterials for bone-regeneration

Billstrom Gry Hulsart , Dawson Jon , Hofmann Sandra , Muller Ralph , Stoddart Martin , Alini Mauro , Redl Heinz , Haj Alicia El , Brown Robert , Salih Vehid , Hilborn Jons , Larsson Sune , Oreffo Richard

Introduction: Research on biomaterials for bone regeneration generates a plethora of new biomaterials requiring evaluation with reliable and comparable methods of biocompatibility and functionality for clinical translation. To reduce the burden of in vivo assessment, there is a need for refined in vitro assays that are predictive of in vivo outcomes. This retrospective study correlated in vitro results with in vivo outcomes observed...

ba0005p84 | Bone Matrix | ECTS2016

Anabolic parathyroid hormone (PTH) treatment does not alter periosteal bone mineral composition during primary and secondary mineralisation

Vrahnas Christina , Pearson Thomas A , Brunt Athena R , Forwood Mark R , Bambery Keith R , Tobin Mark J , John Martin T , Sims Natalie A

Parathyroid hormone (PTH) is used to stimulate bone formation in osteoporotic patients, however concerns have been raised about the quality of the matrix produced since lower levels of total matrix mineral have been reported in osteoporotic and fracture patients treated with PTH. High resolution synchrotron-based Fourier Transform Infrared Microscopy (sFTIRM) was used to determine mineral content in age-matched bone during anabolic PTH treatment, using the simplified lamellar ...

ba0005p274 | Nutrition | ECTS2016

Caffeine at moderate dose did not affect the skeletal system of rats with streptozotocin-induced metabolic disorders

Folwarczna Joanna , Janas Aleksandra , Cegiela Urszula , Pytlik Maria , Sliwinski Leszek , Matejczyk Magdalena , Nowacka Anna , Rudy Karolina , Krivošikova Zora , Štefikova Kornelia , Gajdoš Martin

Diabetes leads to development of osteoporosis. Experimental type 1 diabetes may be induced by a single dose of streptozotocin (STZ), and nicotinamide (NA) administered 15 min before STZ dose-dependently protects against the STZ action. Coffee drinking, apart of its health benefits, is taken into consideration as an osteoporosis risk factor. Data from human and experimental studies on coffee and caffeine effects on the skeletal system are inconsistent. For example, although oth...

ba0005p352 | Osteoporosis: pathophysiology and epidemiology | ECTS2016

Atypical femur fractures (AFF): a case-control study

Imel Erik , Eckert George , Allen Katie , Chandler Julie , Martin Joel , Hui Siu , Johnston C Conrad , DePapp Anne , Santora Art , Choplin Robert , Roth Trenton , Liu Ziyue

Atypical femur fractures (AFF) have been associated with antiresorptive therapy. In a retrospective case-control study, we identified AFF using ASBMR 2013 criteria. Femoral shaft fractures were identified using ICD9 codes. We screened 1479 radiographs. Radiographs were excluded for high-energy trauma, tumor, or periprosthetic fracture. Two radiologists blinded to treatment scored 482 radiographs for AFF features, and jointly adjudicated discrepancies. The required AFF feature,...

ba0006oc26 | (1) | ICCBH2017

A randomized, open-label Phase 2 study of KRN23, an investigational fully human Anti-FGF23 monoclonal antibody, in children with X-linked Hypophosphatemia (XLH)

Hogler Wolfgang , Portale Anthony , Imel Erik , Boot Annemieke , Linglart Agnes , Padidela Raja , van't Hoff William , Whyte Michael , Mao Meng , Skrinar Alison , Martin Javier San , Carpenter Thomas

Objectives: In XLH, FGF23-mediated hypophosphatemia leads to defective bone mineralization and rickets. Investigational product KRN23 binds FGF23 and inhibits its activity. The objective of this Phase 2 study was to evaluate the safety and efficacy of KRN23 in 52 children with XLH (ages 5–12 years, ≤Tanner 2).Methods: Patients were randomized to receive KRN23 biweekly (Q2W) or monthly (Q4W) by SC injection. KRN23 dose was titrated (maximum 2 m...

ba0006p063 | (1) | ICCBH2017

Effects of KRN23, a fully human anti-FGF23 monoclonal antibody, on functional outcomes in children with X-linked hypophosphatemia (XLH): results from a randomized, open-label Phase 2 study

Imel Erik , Carpenter Thomas , Linglart Agnes , Boot Annemieke , Hogler Wolfgang , Padidela Raja , van't Hoff William , Portale Anthony , Mao Meng , Skrinar Alison , San Martin Javier , Whyte Michael P

Objectives: In XLH, musculoskeletal outcomes of current treatment with oral phosphate (Pi)/active vitamin D are suboptimal for many patients. In a Phase 2, open-label study, we tested the hypothesis that KRN23 improves rickets and functional outcomes in XLH children.Methods: Fifty-two children with XLH (ages 5–12 years at baseline) received KRN23 subcutaneously biweekly (Q2W) or monthly (Q4W). At study entry, most participants had received oral Pi/a...

ba0007oc15 | (1) | ICCBH2019

Sustained efficacy and safety of burosumab, a fully human anti-FGF23 monoclonal antibody, in children and early adolescents with X-linked hypophosphatemia

Hogler Wolfgang , Carpenter Thomas , Imel Erik , Portale Anthony , Boot Annemieke , Linglart Agnes , Padidela Raja , Hoff William van't , Mao Meng , Skrinar Alison , Martin Javier San , Whyte Michael

Objective: We evaluated the long-term efficacy of burosumab, a monoclonal antibody against FGF23, in a Phase 2 Study (NCT02163577) in children with XLH.Methods: Fifty-two children with XLH (5-12 years-old, Tanner ≤ 2) were randomized 1:1 to receive subcutaneous burosumab Q2W or Q4W for 64 weeks. Doses were titrated up to 2 mg/kg/dose targeting serum phosphorus levels within 1.1–1.6 mmol/l. All subjects entered the long-term extension at Week 6...