Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p78 | (1) | ICCBH2015

Bone metabolism in children and adolescents with newly diagnosed acute lymphoblastic leukemia

Vai Silvia , Minghetti Sara , Broggi Francesca , Scicchitano Barbara , Sala Alessandra , Corso Rocco , Bianchi Maria Luisa

In children and adolescents with acute lymphoblastic leukemia (ALL), low bone mineral density (BMD) and increased risk of fractures can be observed at diagnosis and/or during treatment.This prospective study was aimed to evaluate BMD and bone turnover in patients with a new diagnosis of ALL, treated with an international protocol (AIEOP BFM ALL 2009) (*), based on high-dose steroids and chemotherapeutic drugs.Inclusion criteria wer...

ba0004p83 | (1) | ICCBH2015

RANKL, OPG, Dkk1 in Duchenne muscular dystrophy

Broggi Francesca , Vai Silvia , Morandi Lucia , Gorni Ksenija , D'Angelo Grazia , Pane Marika , Bianchi Maria Luisa

Low bone mineral density (BMD) and an increased rate of both peripheral and vertebral fractures have been observed in patients with Duchenne muscular dystrophy (DMD). However, studies specifically addressing bone metabolism, BMD and fractures in this disease are still very few.Our ongoing multicenter, prospective study is aimed to identify the characteristics of the DMD boys with a higher risk of bone loss and fractures, through the evaluation of bone tu...

ba0005oc4.1 | Catabolism and metabolism | ECTS2016

A small molecule inhibitor of TRAF6 dependent signaling reduces osteoclastogenesis and prevents ovariectomy induced bone loss

Marino Silvia , Bishop Ryan , Bendle Gavin , Nicolaes Gerry , Lutgens Esther , Idris Aymen

Tumour necrosis factor receptor-associated factor 6 (TRAF6) plays a key role in osteoclastogenesis through the regulation of RANK/CD40 TRAF6-mediated signaling. Mice deficient in TRAF6 exhibit high bone mass and were protected against inflammation-induced bone loss. Here we describe the effects of a small-molecule 6877002 that has been shown to prevent the binding of TRAF6 to its domain on RANK/CD40 receptor on osteoclast formation in vitro and in vivo and on...

ba0005oc5.6 | Risk factors for fracture, Pagets disease of bone and musle and bone | ECTS2016

The metabolic alterations behind bone fragility in Duchenne muscular dystrophy

Broggi Francesca , Vai Silvia , Maggi Lorenzo , Gorni Ksenija , D'Angelo Grazia , Pane Marika , Bianchi Maria Luisa

Low bone mineral density (BMD) and an increased rate of both peripheral and vertebral fractures have been observed in patients with Duchenne muscular dystrophy (DMD), but studies on bone metabolic alterations in this disease are still very few.We are now presenting the preliminary findings of an ongoing multicenter, prospective study aimed to identify the characteristics of DMD boys carrying a higher risk of bone loss and fractures, through the evaluatio...

ba0005p120 | Cancer and bone: basic, translational and clinical | ECTS2016

The role of acidic microenvironment in the context of osteolytic carcinomas

Di Pompo Gemma , Lemma Silvia , Canti Lorenzo , Gillies Robert , Baldini Nicola , Avnet Sofia

The microenvironment of osteolytic metastases includes carcinoma cells derived from the primary lesion as well as bone-forming and bone-resorbing cells, namely osteoblasts (OB) and osteoclasts (OC). At this site, both the high glycolysis of cancer cells, and the bone resorption process result in a very acidic milieu. This, in turn, induces the surrounding stroma and OB to secrete pro-inflammatory cytokines and growth factors that promote tumorigenesis and cancer-associated ost...

ba0005p327 | Osteoporosis: pathophysiology and epidemiology | ECTS2016

Gene expression profiling of osteoblastic cells cultured with lithocholic acid or bilirubin. Implications in the pathogenesis of osteoporosis in liver diseases

Ruiz-Gaspa Silvia , Pares Albert , Dubreuil Marta , Combalia Andres , Peris Pilar , Monegal Ana , Guanabens Nuria

Low bone formation is considered to be the main feature in osteoporosis associated with cholestatic and end-stage liver diseases. Previous studies have demonstrated the deleterious consequences of retained substances such as lithocholic acid (LCA) and bilirubin (Bil) on osteoblastic cells. These effects are neutralized by ursodeoxycholic acid (UDCA). To gain new insights into cholestatic-induced osteoporosis, we have assessed the differential gene expression of osteoblastic ce...

ba0005p482 | Paediatric bone disease | ECTS2016

Body composition in children and young patients affected by chronic diseases

Vai Silvia , Bianchi Maria Luisa , Colombo Carla , Ghio Luciana , Corona Fabrizia , Baranello Giovanni

We analyzed body composition (DXA, Hologic) in 334 young patients (aged 3–24 years) with chronic diseases, most of them on long-term glucocorticoid (GC) treatment, and monitored its changes over 3–14 years (6.9±6.2 years).Bone mineral content (BMC), fat mass (FM) and fat-free mass (FFM) were measured on total body (TB), trunk, upper limbs, lower limbs. BMC, FM, FFM were expressed as percentages and compared with age- and sex-matched health...

ba0005p489 | Preclinical and ex vivo imaging | ECTS2016

Quantitative assessment of radial bone structural distribution in the proximity of degradable implants by micro-computed tomography

Damm Timo , Barkmann Reinhard , Galli Silvia , Willumeit-Romer Regine , Gluer Claus-Christian

Degradable bone implants may provide improved long-term healing, tissue remodeling and quality of life. In order to optimize alloy composition and degradation properties, in-vivo monitoring of the degradation process and its impact on bone formation is essential because degradation progresses differently in-vivo versus in-vitro. We aim to understand how degradation of magnesium alloy implants influences bone remodeling and bone structure using Micro-...

ba0007is10 | (1) | ICCBH2019

Endoplasmic reticulum stress in osteoblasts

Besio Roberta , Tonelli Francesca , Garibaldi Nadia , Leoni Laura , Cotti Silvia , Forlino Antonella

Bone tissue homeostasis requires the coordinated activity of osteoblasts, the bone forming cells, of osteoclasts, the bone resorbing cells, and of osteocytes, generally referred as the bone mechano-sensors. In this contest, osteoblasts are the mesenchymal cells secreting the extracellular matrix components on which hydroxyapatite crystals are then deposited. The most abundant protein of this organic matrix is type I collagen, a heterotrimeric secretory protein, synthesized as ...

ba0007p36 | (1) | ICCBH2019

ALPL gene mutation in a family

Vai Silvia , Broggi Francesca , Luisa Bianchi Maria , Ponti Emanuela , Mihalich Alessandra , Maria Di Blasio Anna

Introduction: The clinical diagnosis of mild forms of hypophosphatasia [HPP], a rare genetic bone disease, is often made in adulthood, on the basis of persistently low serum levels of alkaline phosphatase [ALP], often coupled with signs of poor bone/teeth mineralization.Case report: A 50-year-old male on treatment with vitamin D supplementation because of osteoporosis of lumbar spine (T-score –3.2) and femoral neck (T-score –2.4), was referred ...