Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p115 | (1) | ICCBH2019

Mabry Syndrome is a cause of hyperphosphatasia and mental retardation

Tseretopoulou Xanthippi , Mughal Zulf , Mushtaq Talat

Background: Hyperphosphatasia may be seen in liver disorders or metabolic bone disease with the most common cause likely to be Vitamin D deficiency. However, we report the case of child who had high ALP levels from infancy along with intellectual retardation. Genetic testing revealed Mabry Syndrome.Presenting problem: At birth, a micrognanthia and a cleft palate was apparent. She went to have developmental delay, impaired vision, and was wheelchair depen...

ba0007p140 | (1) | ICCBH2019

Safety and effectiveness of stoss therapy in children

Fiscaletti Melissa , Tannous Paul , Wood Nicholas , Gunasekera Hasantha , Zurynski Yvonne , Biggins Andrew , Kilo Tatjana , Hayes Evan , Munns Craig

Objectives: Pediatric vitamin D (25-hydroxyvitamin D - 25OHD) deficiency can lead to nutritional rickets and extra-skeletal complications. Compliance with daily therapy can be difficult, making high dose, short-term vitamin D (stoss) therapy attractive to correct vitamin D deficiency. We compared the effectiveness and safety of standard versus stoss therapy in treating childhood 25OHD deficiency.Study design: Children aged 2–16 years with 25OHD &#60...

ba0007p169 | (1) | ICCBH2019

Rescue diet restores bone matrix mineralization in mice with a non-functioning vitamin D receptor

Misof Barbara , Blouin Stephane , Hartmann Markus , Hofstaetter Jochen , Klaushofer Klaus , Zwerina Jochen , Roschger Paul , Erben Reinhold

Objectives: Mice with a non-functioning vitamin D receptor (VDR mutants) develop severe secondary hyperparathyroidism, which can be rescued by a diet enriched with calcium, phosphate and lactose. In this work, we studied the effects of a low calcium challenge (CD), normal calcium (ND) and a calcium enriched rescue diet (RD) on the bone mineralization density distribution (BMDD) and osteocyte lacunae sections (OLS) in these mice.Methods: BMDD and OLS were...

ba0007p223 | (1) | ICCBH2019

A preliminary data of a prospective study on Iranian patients with osteogenesis imperfecta

Hoseinbeyki Moslem , Moradifard Shirin , Mirkhani Fatemeh , Ehsani Parastoo , Saghiri Reza , Karimipoor Morteza , Alaei Mohammareza , Ebrahimi-Rad Mina

Objectives: Osteogenesis Imperfecta (OI), is a group of rare, heritable disorder of bone and connective tissue. The pathogenicity of OI arises from the mutations in about 17 different genes, involved in collagen type 1 synthesis, processing, post-translational modification, folding, cross-linking, bone mineralization, and osteoblast differentiation. Based on Sillence classification, there are four types of OI; Type I (mild, non-deforming), Type II (perinatal lethal), Type III ...

ba0001oc4.2 | Osteoblasts and osteocytes | ECTS2013

The p38α MAPK pathway in osteoblasts contributes to ovariectomy-induced bone loss by upregulating interleukin 6 expression

Thouverey Cyril , Caverzasio Joseph

Selective p38α inhibitors have been found to prevent bone loss induced by estrogen deficiency but implicated mechanisms remained to be identified. The p38 MAPK pathway has been suggested to influence bone resorption at different regulatory levels. In osteoblasts, p38α has been reported to be involved in the production of osteoclastogenic interleukin 6 and Rankl in response to various bone-resorptive agents in vitro. Therefore, we investigated whether p38&#94...

ba0001pp32 | Bone biomechanics and quality | ECTS2013

Vitamin D levels of >85 nmol/l in the presence of adequate dietary Ca minimise bone turnover and improve bone strength

O'Loughlin Peter , Lee Alice , Anderson Paul , Steck Roland , Forwood Mark , Sawyer Rebecca , Morris Howard

We have reported femoral osteopenia in short term-vitamin D restricted rats without deterioration in tibial cortical bone volume (CBV), geometry or strength1. This study aimed to establish the effect of extended vitamin D deficiency in aged rat tibial volume and strength. Female Sprague-Dawley rats (9 m, n=6/group) were fed a diet containing varying vitamin D3 (D) levels (0, 2, 12, and 20 IU/day) with either low (0.1%, LCa) or high (1%, HCa) diet...

ba0001pp58 | Bone biomechanics and quality | ECTS2013

Diagnostic discrimination of TBS and spine BMD in glucocorticoid-induced and postmenopausal osteoporosis

Paggiosi Margaret , Peel Nicola , Eastell Richard

Glucocorticoids inhibit osteoblast function and cause an increase in osteoblast and osteocyte apoptosis. Bone remodelling defects occur resulting in an increase in fracture risk that cannot be fully explained by decreases in bone mineral density (BMD). We propose that this may be due to alterations in bone quality. Trabecular bone score (TBS) correlates with 3D bone micro-architectural parameters and can be derived directly from grey-level variations within 2D DXA images.<...

ba0001pp189 | Cell biology: osteoblasts and bone formation | ECTS2013

Integrins and cadherins in mesenchymal stem cells from dental tissues: possible implication in the osteogenic differentiation process

Di Benedetto Adriana , Carbone Claudia , Oranger Angela , Brunetti Giacomina , Lorenzo Lo Muzio , Colucci Silvia , Grano Maria , Mori Giorgio

Numerous studies have reported beneficial effects of multipotent mesenchymal stem cells (MSCs) in tissue repair and regeneration. These multipotent cells can be isolated from many different adult tissues and give rise to different cell lineages. The most well-characterized source for adult stem cells is still adult bone marrow, however in the past decade, subpopulations of stem cells have been isolated from dental tissues. Dental pulp has been identified as a promising source ...

ba0001pp311 | Osteoporosis: evaluation and imaging | ECTS2013

Appropriate osteoporosis treatment by family physicians in response to FRAX vs caroc reporting: a randomized controlled trial

Beattie Karen , Ioannidis George , MacDermid Joy , Grewal Ruby , Papaioannou Alexandra , Hodsman Anthony , Adachi Jonathan D

Introduction: Current Canadian clinical practice guidelines recommend the FRAX or Canadian Association of Radiologists and Osteoporosis Canada (CAROC) fracture risk assessment tools to report 10-year fracture risk in an individual. CAROC considers sex, age, BMD and previous fracture as risk factors. It is unknown whether one reporting system is more effective in helping general practitioners (GPs) identify individuals who should be recommended for pharmacological treatment. We...

ba0002oc1 | Epidemiology | ICCBH2013

The Amalgamated Paediatric Bone Density Study (The ALPHABET Study): the collation and generation of UK based reference data for paediatric bone densitometry

Crabtree Nicola , Machin Mike , Bebbington Natalie , Adams Judith , Ahmed Faisal , Arundel Paul , Bishop Nicholas , Fewtrell Mary , Hogler Wolgang , Mughal M Zulf , Rhodes Laura , Shaw Nicholas , Ward Kate

Understanding normal patterns of bone growth is important for optimising bone health in children and reducing osteoporotic fractures in later life. Recently published guidelines for bone assessment in children state that to predict fractures a technique should identify children at risk of clinically significant fractures and that dual-energy absorptiometry (DXA) is the preferred method of assessment. Despite these guidelines there is still inconsistency and lack of consensus r...