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...

ba0001pp375 | Osteoporosis: pathophysiology and epidemiology | ECTS2013

25-Hydroxyvitamin D values in liver transplant candidates

Monegal Ana , Peris Pilar , Cuervo Andrea , Muxi Africa , Gifre Laia , Guanabens Nuria

Introduction: Liver transplant candidates have bone and mineral metabolism disorders that may influence the development of fractures after liver transplantation (LT).Objective: To analyze the levels of 25-hydroxyvitamin D (25-OH-D) in patient candidates for LT and the factors associated with vitamin D deficiency.Methods: Between January 2010 and May 2012, 116 liver transplant candidates (85 male and 31 female patients) were include...

ba0002p191 | (1) | ICCBH2013

Long-term follow-up in Stuve–Wiedemann syndrome: a case report

Sabrina Buonuomo Paola , Macchiaiolo Marina , Cambiaso Paola , Capolino Rossella , Digilio Maria Cristina , Andrea Bartuli

Background: Stuve–Wiedemann syndrome (SWS, OMIM 601559) is a severe autosomal recessive condition characterized by bowing of the lower limbs with cortical thickening, wide metaphyses, abnormal trabecular pattern and camptodactyly. Additional features include dysautonomia symptoms with temperature instability, respiratory distress and sucking/swallowing difficulties in the first months of life.Most SWS cases do not survive beyond the first y...

ba0003pp145 | Cell biology: osteoblasts and bone formation | ECTS2014

Effects of strontium ranelate on the bone-like mineralized matrix produced in osteoblast cell cultures

Querido William , Campos Andrea , Ferreira Erlon Martins , Gil Rosane San , Rossi Alexandre , Farina Marcos

Strontium ranelate is a promising drug used in the treatment of osteoporosis. This drug has a unique dual effect on bone turnover, simultaneously increasing bone formation by osteoblasts (anabolic effect) and decreasing bone resorption by osteoclasts (antiresorptive effect). The goal of this study was to evaluate: i) anabolic effects of strontium ranelate on the formation of bone-like mineralized matrix in osteoblast cell cultures and ii) changes the drug could cause on the ma...

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...

ba0003pp164 | Cell biology: osteoclasts and bone resorption | ECTS2014

Novel highly sensitive ELISA to measure free, bioactive, human soluble RANKL

Suciu Andreea , Breitwieser Andreas

RANKL, the receptor activator of nuclear factor kappa B ligand, is an essential factor for the formation of mature osteoclasts. Together with its receptor RANK and its antagonist Osteoprotegerin (OPG) RANKL is a key regulator in bone metabolism1. RANKL is a membrane-bound protein that can be segregated to a soluble form (sRANKL), whereas only the latter has been reported to be bioactive2. Due to its low circulating levels and the nature of the analyte bin...

ba0001pp435 | Osteoporosis: treatment | ECTS2013

Denosumab's dynamic CTX profile is maintained over 6 years of treatment: first 3 years of the FREEDOM extension study

Roux C , McClung MR , Franchimont N , Adami S , Ebeling PR , Reid IR , Resch H , Weryha G , Daizadeh N , Wang A , Wagman RB , Eastell R

Denosumab (DMAb) has a unique profile of bone resorption inhibition: CTX decreases rapidly by 3 days and inhibition is released at the end of the 6-month dosing interval, when DMAb serum levels decrease (McClung NEJM 2006). The dynamic CTX inhibition profile is not curtailed by continued treatment. In the 3-year FREEDOM study, CTX values at 6 months were influenced by baseline CTX values and days since the 1st injection (Eastell JBMR 2011). With 3 additional ...