Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp73 | Bone development/growth and fracture repair | ECTS2013

Feasibility of local CD133+ cell transplantation to avoid non-unions in biological impaired bone healing

Dienelt Anke , Sass Andrea F , Preininger Bernd , Schmidt-Bleek Katharina , Duda Georg N

The clinical orthopaedic problem of delayed healing or non-union after complex fractures affects 5–10% of all patients, especially within the elderly population. Recently several in vitro studies showed that CD133+ cells bare angiogenic capacities and contribute to a better outcome concerning ischemia induced angiogenesis in vivo. A local administration of these specific cells to the fracture gap appears feasible as a new treatment option for biological ...

ba0001pp357 | Osteoporosis: pathophysiology and epidemiology | ECTS2013

The prevalence of osteoporosis and risk factors for bone demineralization in Italy: first results from the firmo study

Fossi Caterina , Cavalli Loredana , Giusti Francesca , Metozzi Alessia , Parri Simone , Guazzini Andrea , Maria Luisa Brandi

Objective(s): We aimed to determine the prevalence of osteoporosis and risk factors for bone demineralization in the Italian population.Material and methods: 3090 consecutive subjects were screened for osteoporosis by using calcaneal quantitative ultrasounds (QUS) in 16 Italian cities (women: 2635; men: 455) during the extension of the FIRMO study carried out in 2011 on about 7000 people. Anamnestic data were collected to assess the presence of recognize...

ba0004p73 | (1) | ICCBH2015

Long-term outcomes of surgical treatment for craniofacial fibrous dysplasia

Boyce Alison , Burke Andrea , Peck Carolee Cutler , Dufresne Craig , Collins Michael

Fibrous dysplasia (FD) is a benign skeletal disease caused by somatic activating mutations of Gsα leading to formation of expansile fibroosseous lesions. These may occur in isolation or in association with McCune-Albright syndrome (MAS), characterized by skin pigmentation and hyperfunctioning endocrinopathies, including growth hormone (GH) excess. FD in the craniofacial skeleton may result in significant morbidity including facial asymmetry, vision and hearing ...

ba0004p158 | (1) | ICCBH2015

Extreme, biomechanically-explained remodelling of biological femoral reconstructions in pediatric oncology

Taddei Fulvia , Valente Giordano , Piroddi Sabina , Schileo Enrico , Pitto Lorenzo , Roncari Andrea , Leardini Alberto , Manfrini Marco

Introduction: Vascularised fibula autograft combined with a massive bone allograft (Capanna 2007) is used in skeletal reconstructions in children. If vascularisation is successful a clear remodelling of the reconstruction can be observed. This study aims to define a protocol to characterise bone’s structural evolution in skeletal reconstructions through a computer-aided analysis, and attempts a biomechanical interpretation of the observed phenomena through a multiscale mo...

ba0006p039 | (1) | ICCBH2017

Cystinosin deficiency affects bone phenotype

Battafarano Giulia , Rossi Michela , Di Giovamberardino Gianna , Pastore Anna , Taranta Anna , Del Fattore Andrea

Objective: Cystinosis is a rare lysosomal storage disorder caused by loss-of-function mutations of the CTNS gene, encoding for cystinosin symporter that mediates cysteine efflux from lysosome. ~95% of cystinotic patients display nephropathic Fanconi’s syndrome, short stature, osteopenia and rickets. In this study we evaluated whether the absence of cystinosin primarily affects bone remodeling activity.Methods: We analyzed bone phen...

ba0001pp188 | Cell biology: osteoblasts and bone formation | ECTS2013

Intracellular calcium fluxes in human bone cells in osteoporotic and osteoarthritic patients

celi Monica , Gasbarra Elena , Frank Claudio , Cutarelli Alessandro , Cinci Giulio Fioravanti , Tarantino Umberto

We studied changes in intracellular Ca2+ concentration in bone cell cultures obtained from human subjects with osteoporosis and osteoarthritis, to evaluate differences between these patients and healthy subjects. We enrolled 36 patients: 12 undergoing primary total hip arthroplasty for osteoporotic femoral fractures (group A, mean age range 57–80), 12 for hip osteoarthritis (group B, mean age range 57–80), and 12 healthy subjects who suffered a high-energy...

ba0005p413 | Osteoporosis: treatment | ECTS2016

Dose postoperative applications of diphosphonate medicine at an early stage have influence on union of fracture?

Li Qiang , Zhang Jia , Zhu Shen

Background: Surgical and conservative treatments are both crucial method to deal with osteoporotic fracture, anti-osteoporotic therapy is the most important among conservative treatments and diphosphonate medicine is the first-line choice. The pharmacological action of diphosphonate medicine is to inhibit the function of osteoclast, so there is no consensus if it will inhibit porosis and union of fracture.Purpose: To find out the influence of diphosphona...

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...

ba0006lb3 | (1) | ICCBH2017

Mediating effect of muscle on the relationship of physical activity trajectories and bone outcomes: The Iowa Bone Development Study

Zymbal Vera , Baptista Fatima , Letuchy Elena M. , Janz Kathleen F.

Objectives: This study analysed prospective associations between two distinct developmental trajectories of objectively-measured physical activity and late adolescent bone parameters (age 17 yr) by exploring the mediating effects of lean soft tissue (LST), a surrogate of muscle mass.Methods: In approximately 349 participants (191 girls) of the Iowa Bone Development Study, physical activity was measured by accelerometry starting at age 5 and continuing at...

ba0004is15biog | (1) (1) | ICCBH2015

Achondroplasia-new therapy

Legeai-Mallet Laurence

Biographical DetailsLaurence Legeai-Mallet is currently Director of Research at Imagine Institute-Paris Descartes University. She received her PhD in genetic from University of Paris V, she is a member of International skeletal dysplasia Society, European Skeletal Dysplasia Network and the French reference center of bone dysplasias. She has been involved in the field of skeletal disease s...