Searchable abstracts of presentations at key conferences on calcified tissues

ba0003pp371 | Other diseases of bone and mineral metabolism | ECTS2014

Effect of complete spinal cord injury on bone turnover and bone mineral density evolution: a 1 year follow-up study

Gifre Laia , Vidal Joan , Ruiz-Gaspa Silvia , Portell Enric , Monegal Ana , Muxi Africa , Guanabens Nuria , Peris Pilar

Spinal cord injury (SCI) has been associated with marked bone loss and an increased risk of fractures under the SCI level. The aim of the study was to analyze the effect of recent complete SCI on bone turnover and bone mineral density (BMD) evolution and factors related to bone loss.Methods: Prospective study including patients with complete motor SCI (ASIA A or B) (<6 months). Bone turnover markers (bone formation: P1NP, bone ALP and resorption: sCT...

ba0005p121 | Cancer and bone: basic, translational and clinical | ECTS2016

MCT1 as a novel target for the treatment of osteolytic bone metastases

Avnet Sofia , Lemma Silvia , Di Pompo Gemma , Sboarina Martina , Porporato Paolo , Perez-Escuredo Jhudit , Sonveaux Pierre , Baldini Nicola

Bone metastasis (BM) is a dismal complication of cancer, occurring frequently in patients with advanced breast carcinoma. During metastatic progression, carcinoma cells harness osteoclast (OC) activity, promoting osteolysis. To adapt to hypoxia and/or to support proliferation, carcinoma cells adopt primarily glycolysis for energy production, therefore releasing lactic acid in the microenvironment through monocarboxylate transporter 4 (MCT4). Stressed by tumor cells, osteoblast...

ba0005p127 | Cancer and bone: basic, translational and clinical | ECTS2016

LIGHT promotes osteolytic bone metastases in NSCLC patients

Brunetti Giacomina , Belisario Dimas Carolina , Alliod Valentina , Buffoni Lucio , Colucci Silvia , Grano Maria , Ferracini Riccardo , Roato Ilaria

LIGHT is a TNF superfamily member, expressed by activated T cells. It is involved in erosive bone disease, such as rheumatoid arthritis where it stimulates osteoclastogenesis. In multiple myeloma, LIGHT promotes osteolysis by increasing osteoclastogenesis and inhibiting osteoblastogenesis. We investigated whether LIGHT has a role in the osteolytic bone metastatic process induced by non small cell lung cancer (NSCLC), which is a tumor with a marked osteotropism. We analysed by ...

ba0005p366 | Osteoporosis: pathophysiology and epidemiology | ECTS2016

Effect of recent spinal cord injury on the OPG/RANKL system and its relationship with bone loss and antiosteoporotic response to denosumab therapy: preliminary results

Gifre Laia , Vidal Joan , Ruiz-Gaspa Silvia , Portell Enric , Monegal Ana , Muxi Africa , Guanabens Nuria , Peris Pilar

The aims of this study were to analyze the role of the regulators of bone remodeling, OPG and RANKL, in the bone loss associated with recent spinal cord injury (SCI) as well as the effect of antiosteoporotic therapy with denosumab in these bone regulators in a prospective study.Patients and methods: Twenty-three male patients (aged 18–67 years (mean 36±16 years)) with recent (<6 months) complete SCI were prospectively included (43.5% parapl...

ba0006p053 | (1) | ICCBH2017

Determinants of bone density in Duchenne muscular dystrophy

Broggi Francesca , Vai Silvia , Baranello Giovanni , Gorni Ksenja , D'Angelo Grazia , Pane Marika , Vita Gianluca , Bianchi Maria Luisa

Objectives: Low bone mineral density (BMD) and increased frequency of peripheral and vertebral fractures have been reported in boys with Duchenne muscular dystrophy (DMD), but studies on the determinants of low BMD are still very few. We are currently carrying out a multicenter, prospective study aimed to identify the characteristics of DMD boys with a higher risk of bone loss and fractures.Methods: Forty-two DMD boys (mean age 9.9±3.3 years) underw...

ba0007p28 | (1) | ICCBH2019

Duchenne muscular dystrophy: preliminary results of the Risbo-DMD study

Broggi Francesca , Vai Silvia , Baranello Giovanni , Sansone Valeria Sansone , D'Angelo Grazia , Pane Marika , Vita Gianluca , Bianchi Maria Luisa

Introduction: Reduced bone mineral density [BMD] and increased fracture risk are common complications in all conditions characterized by severely reduced physical activity and/or requiring long-term glucocorticoid [GC] treatment, including Duchenne Muscular Dystrophy [DMD].Objectives: The RisBo-DMD study (EudraCT 2011-005745-12) is a 24-month prospective multicenter study, aimed at identifying DMD patients at higher risk of fractures and improving the bo...

ba0007p37 | (1) | ICCBH2019

Generation of osteogenesis imperfecta type XIV zebrafish models

Leoni Laura , Tonelli Francesca , Cotti Silvia , Giannini Gabriella , Daponte Valentina , Gioia Roberta , Besio Roberta , Garibaldi Nadia , Rossi Antonio , Forlino Antonella

Objectives: Osteogenesis Imperfecta (OI) type XIV is a recessive OI form characterized by bone fragility, multiple fractures and growth retardation. It is caused by mutation in TMEM38B gene encoding the endoplasmic reticulum (ER) channel TRIC-B. This channel allows the transport of K+ across the ER membrane modulating Ca2+ flux. Defective ER Ca2+ impaires collagen type I synthesis, likely affecting the activity of ER enzymes involved in its post translational modification. To ...

ba0001pp146 | Cancer and bone: basic, translational and clinical | ECTS2013

Metabolomics identifies plasma biomarkers of multiple myeloma development and progression

Mariani Elisabetta , Fontana Francesca , Mari Silvia , Manteiga Jose Manuel Garcia , Marcatti Magda , Napoli Nicola , Camnasio Francesco , Fraschini Gianfranco , Caneva Enrico , Sitia Roberto , Musco Giovanna , Ciceri Fabio , Cenci Simone

Multiple myeloma is an incurable neoplastic disorder of plasma cells, which invade the bone marrow, secrete monoclonal immunoglobulins, and induce bone lesions, hypercalcemia, anemia and renal failure. The development of myeloma relies on vicious interactions with the bone microenvironment, a deeper knowledge of which is needed to identify prognostic markers and potential therapeutic targets. To achieve an unbiased, comprehensive assessment of the extracellular milieu of myelo...

ba0001pp503 | Paediatric bone disease | ECTS2013

High dickkopf-1 levels in sera and leukocytes from children with 21-hydroxylase deficiency on chronic glucocorticoid treatment

Brunetti Giacomina , Maria Felicia Faienza , Piacente Laura , Ventura Annamaria , Oranger Angela , Carbone Claudia , Benedetto Adriana Di , Colaianni Graziana , Mori Giorgio , Colucci Silvia , Cavallo Luciano , Grano Maria

Children with 21-hydroxylase deficiency (21-OHD) need chronic glucocorticoid (cGC) therapy to replace congenital deficit of cortisol synthesis, and this therapy is the most frequent and severe form of drug-induced osteoporosis. In the study we enrolled 18 patients (9 females) and 18 sex- and age-matched controls. We found in 21-OHD patients high serum and leukocyte levels of dickkopf-1 (DKK1), a secreted antagonist of the Wnt/β-catenin signaling pathway, known to be a key...

ba0002op2 | (1) | ICCBH2013

High FSH serum levels may support the altered bone remodeling in Turner syndrome patients

Brunetti Giacomina , Ventura Anna Maria , Piacente Laura , Oranger Angela , Ciccarelli Maria , Mori Giorgio , Colucci Silvia , Cavallo Luciano , Grano Maria , Faienza Maria Felicia

Objective: Turner syndrome (TS) is a chromosomal aberration characterized by total or partial loss of one of the two X-chromosomes, and affects about 1 in every 2500 girls. TS patients can develop the bone disease with decreased bone density and selective reduction in cortical bone thickness, which probably contributes to the increased fracture risk. However, the mechanisms underlying the bone disease remain poorly understood. Thus, the aim of this study was to investigate the...