Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp82 | Bone development/growth and fracture repair | ECTS2013

Measurement properties of radial and tibial speed of sound for screening bone health and fragility in 10–12 years old boys and girls

Rebocho Lurdes , Cardadeiro Graca , Zymbal Vera , Goncalves Ezequiel M , Sardinha Luis B , Baptista Fatima

The objective of this study was to analyze measurement properties of BeamMed Omnisense quantitative ultrasound (QUS) of the radial and tibial speed of sound (SoS) for assessing bone health and screening bone fragility in youth. Bone fragility was defined as low whole body less head bone mineral density (WBLH BMD) measured by DXA (first tertile, 95% CI: −1.1– (−0.9)) and as past history of fractures evaluated by questionnaire. The study was conducted with 319 n...

ba0001pp283 | Genetics | ECTS2013

Discovery and replication of several loci significantly associated with lean body mass: a large meta-analysis of genome wide association studies (GWAS) from the ‘charge’ and ‘gefos’ consortia

Kiel Douglas P , Yerges-Armstrong Laura M , Hsu Yi-Hsiang , Stolk Lisette , Karasik David , Loos Ruth J F , Gudnason Vilmundar , Smith Albert , O'Connell Jeffrey R , Fu Amish , Fu Mao , Streeten Elizabeth A , Cauley Jane A , Robbins John A , Psaty Bruce , Johnson Toby , Kutalik Zoltan , Mitchell Braxton D , Livshits Gregory , Harris Tamara B , Ohlsson Claes , Zillikens M Carola

Introduction: The creatine kinase (CK) is a dimeric enzyme, involved in energetical metabolism. It is present in many tissues, but higher concentration in skeletal and cardiac muscle.Therefore, conditions that involve muscle tissue may increase this serum enzyme. Such enzyme elevation is usually observed in inflammatory myopathies and others autoimmune diseases.Sometimes some elevation in CK is not fully understood out off these co...

ba0006p065 | (1) | ICCBH2017

A novel form of congenital rickets due to a recurrent gain of function mutation in CYP3A4

Rodda Christine P , Levine Michael A , Roizen Jeffrey D , Javaid Muhammad K , Ebeling Peter R , Nguyen Hanh , Dewez Peter , Shaw Nicholas J

Although Vitamin D deficiency is the most common form of rickets worldwide, when there is a failure to respond to cholecalciferol, inborn errors of vitamin D metabolism should be considered. We describe two unrelated individuals who presented with early onset rickets characterised by reduced serum levels of 25(OH)D and 1,25(OH)2D, and a deficient response to Vitamin D2/D3 and calcitriol. Case 1: A Caucasian Australian girl with non-consanguineous parents ...

ba0003pp113 | Cell biology: osteoblasts and bone formation | ECTS2014

Bone-anabolic effects of sulforaphane, a naturally occurring isothiocyanate on bone metabolism

Thaler Roman , Dudakovic Amel , Rucci Nadia , Maurizi Antonio , Sturmlechner Ines , Spitzer Silvia , Rumpler Monika , Van Wijnen Andre J , Teti Anna , Klaushofer Klaus , Varga Franz

Few drugs generate bone-stimulatory effects via epigenetic mechanisms. Modulation of CpG-residues hydroxymethylation in gene-promoters of key osteoblast-related factors (e.g., DLX5) induces their expression and increases osteoblast differentiation in vitro. The chemical properties of sulforaphane (SFN), a natural compound abundantly present in cruciferous vegetables like broccoli, suggest that it may have similar molecular and biological effects. Previous stu...

ba0001pp174 | Cell biology: osteoblasts and bone formation | ECTS2013

Elevated levels of serotonin decrease bone volume by direct effects on bone turnover in rats

Erjavec Igor , Bordukalo-Niksic Tatjana , Brkljacic Jelena , Pauk Martina , Grgurevic Lovorka , Thompson David D , Paralkar Vishwas M , Cicin-Sain Lipa , Vukicevic Slobodan , Mokrovic Gordana , Kesic Maja , Grcevic Danka

Elevated levels of circulating serotonin have been reported to decrease bone mineral density1. Conversely, reduced serotonin (5HT) in mice lacking TPH1, the rate limiting enzyme for 5HT synthesis, was reported to be anabolic to the skeleton with high osteoblastic activity2. However, in other studies TPH1 deletion led to either an initial increase in BMD due to inhibition of osteoclastic bone resorption3, or had no bone effect4</su...

ba0001pp141 | Cancer and bone: basic, translational and clinical | ECTS2013

Therapeutic interest of Imatinib Mesylate in osteosarcoma

Gobin Berengere , Moriceau Gatien , Ory Benjamin , Brion Regis , Redini Francoise , Heymann Dominique

Osteosarcoma is the most common primary malignant bone tumor, characterized by osteoid production and/or osteolytic lesions of bone. A lack of response to chemotherapeutic treatments points out the importance of exploring new therapeutic ways. Imatinib Mesylate (Glivec, Novartis Pharma), a tyrosine kinase inhibitor, has been originally developed for the treatment of chronic myeloid leukemia. Several studies revealed that Imatinib Mesylate inhibits osteoclast differentiation th...

ba0007p46 | (1) | ICCBH2019

Vertebral fractures are more prevalent than long bone fractures in boys with glucocorticoid-treated Duchenne Muscular Dystrophy: Results of a prospective observational study

Jackowski Stefan A , McAdam Laura C , Ma Jinhui , McMillan Hugh J , Konji Victor N , Matzinger Mary Ann , Shenouda Nazih , Jaremko Jacob L , Koujok Khaldoun , Khan Nasrin , Khatchadourian Karine , Robinson Marie-Eve , MacLeay Lynn , Saleh David S , Siminoski Kerry , Rauch Frank , Ward Leanne M

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

ba0006p134 | (1) | ICCBH2017

Improvement in spinal involvement with zoledronic acid in pediatric patients with chronic recurrent multifocal osteomyelitis: a case series

Robinson Marie-Eve , Sbrocchi Anne Marie , Scuccimarri Rosie

Background: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare inflammatory bone disease characterized by chronic non-infectious osteomyelitis. Spinal involvement has been reported in up to 26% of patients (1). Three studies evaluated the effect of Pamidronate (PAM) on spinal lesions in pediatric patients with CRMO (1, 2, 3) and showed partial or complete resolution of vertebral hyperintensities on MRI (1). However, the effect of Zoledronic acid (ZOL) in pediatric pat...

ba0001pp245 | Cell biology: osteocytes | ECTS2013

IGF1 regulates MC-3T3 and human primary osteoblast to osteocyte differentiation in 3D culture

Scully Nicole E E , Mason Deborah J , Evans Bronwen A J

Osteocytes differentiate from osteoblasts, are embedded in mineralised matrix and are critical regulators of bone remodelling. In vitro osteocyte models are limited to cell lines in monolayer, which do not represent their 3D environment in vivo. We have shown that osteoblasts in 3D gels differentiate along the osteocytic pathway. Since IGF1 regulates osteoblasts, and is involved in osteocyte response to mechanical loading, we hypothesised that IGF1 modulates ...