Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p72 | (1) | ICCBH2019

Burosumab experience in UK X-linked hypophosphataemia children under five years old

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone and rickets. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We report relevant real-world biochemical data on children under five years ...

ba0007p73 | (1) | ICCBH2019

Burosumab initiation in a UK X-linked hypophosphataemia cohort: real-world use resonates with research evidence

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone resulting in rickets, skeletal abnormalities, physical impairment, weakness, and pain. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We...

ba0001pp2 | Clinical case posters | ECTS2013

Osteonecrosis of the jaw in a patient with rheumatoid artritis treated with an oral aminobisphosphonate: a clinical case report

Longato Lorena , Cavalli Loredana , Marcucci Gemma , Metozzi Alessia , Giusti Francesca , Brandi Maria Luisa , Piscitelli Prisco

Osteonecrosis of the jaw (ONJ) has been recently described after i.v. administration of amino-bisphosphonates and – less frequently – in association with the use of oral bisphosphonates. Bisphosphonate-related osteonecrosis of the jaw (BRONJ) may affect mandible bone (65%), maxilla bone (26%) and rarely (9%) both sites simultaneously. Although causality may never be proven, emerging experimental data have established a strong association between monthly i.v. bisphosp...

ba0001pp23 | Arthritis and other joint diseases: translational and clinical | ECTS2013

Ultrasound carotid plaque morphology in rheumatoid arthritis women without previous cardiovascular events

Castro Alice , Carmona-Fernandes Diana , Santos Maria Jose , Mendes-Pedro Luis , Canhao Helena , Fonseca Joao Eurico

Introduction: In rheumatoid arthritis (RA) patients subclinical atherosclerosis and cardiovascular events (CV) occur more frequently and at younger ages than in the general population. Previous data suggest that heterogeneous plaques on USA are more unstable and frequently contain a higher amount of lipids and which make them hypoechoic and had higher potential for embolization and thrombosis. The aim of our work was to estimate the prevalence and the ultrasound morphology of ...

ba0001pp124 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2013

Polyphenols from berries of Aronia melanocarpa improve the antioxidative capacity of the bone tissue in cadmium-exposed rats

Rogalska Joanna , Brzoska Malgorzata M , Roszczenko Alicja , Galazyn-Sidorczuk Malgorzata , Jurczuk Maria

Cadmium is characterized by oxidative properties and this heavy metal-induced oxidative stress has been recognized to be involved in its injurious impact on the skeleton. Oxidative/reductive processes are an integral component of bone remodeling; however, destroying of the bone tissue oxidative/antioxidative status with excessive production and accumulation of reactive oxygen species has detrimental impact on bone metabolism. Thus, the aim of this study was to investigate whet...

ba0001pp133 | Cancer and bone: basic, translational and clinical | ECTS2013

Identification of tumorigenic sarcoma cancer stem cells based on high aldehyde dehydrogenase 1 activity

Lohberger Birgit , Rinner Beate , Stuendl Nicole , Walzer Sonja Maria , Windhager Reinhard , Leithner Andreas

Tumors contain a small population of cancer stem cells (CSC) proposed to be responsible for tumor maintenance and relapse. Aldehyde dehydrogenase 1 (ALDH1) activity has been used as a functional stem cell marker to isolate CSCs in different cancer types. This study used the Aldefluor assay and fluorescence-activated cell sorting (FACS) analysis to isolate ALDH1high cells from five human sarcoma cell lines and one primary chordoma cell line. ALDH1high cell...

ba0001pp149 | Cancer and bone: basic, translational and clinical | ECTS2013

Cytotoxicity of picocyanobacteria strains of the genera Cyanobium on osteosarcoma cells

Martins Rosario , Costa Margarida , Garcia Monica , Barros Piedade , Costa-Rodrigues Joao , Vasconcelos Vitor , Fernandes Maria

Marine cyanobacteria have been recognized as an important source of bioactive compounds. The cytotoxicity on cancer cell lines has been extensively explored and several cyanobacteria metabolites are already described as potential anticancer compounds or are considered useful templates for the design of new anticancer drugs. The majority of compounds have been isolated from filamentous or colonial cyanobacteria that growth in high densities along shores. In contrast, picoplankt...

ba0001pp170 | Cell biology: osteoblasts and bone formation | ECTS2013

Calcium transport and phosphomonohydrolase activity by proteoliposomes harboring annexin V and alkaline phosphatase

Bolean Mayte , Simao Ana Maria , Kiffer-Moreira Tina , Hoylaerts Marc , Millan Jose Luis , Ciancaglini Pietro

The biomineralization process is initiated inside matrix vesicles (MVs), with phosphate and calcium ions crystallizing as hydroxyapatite. This process is accomplished by the activities of several proteins, such as annexins (e.g. AnxV) that mediates Ca2+ influx into MVs and tissue-nonspecific alkaline phosphatase (TNAP), a phosphomonohydrolase that uses ATP and PPi as substrates. Dipalmitoylphosphatidylcholine (DPPC) and dipalmitoylphosphatidylserine (DPPS...

ba0001pp219 | Cell biology: osteoclasts and bone resorption | ECTS2013

Negative modulation of human osteoclastogenesis by antiepileptic drugs

Rocha Sara , Costa-Rodrigues Joao , Ferraz Ricardo , Prudencio Cristina , Fernandes Maria

Bone is constantly being molded and shaped by the action of osteoclasts and osteoblasts. A proper equilibrium between both cell types metabolic activities is required to ensure an adequate skeletal tissue structure, and it involves resorption of old bone and formation of new bone tissue. It is reported that treatment with antiepileptic drugs (AEDs) can elicit alterations in skeletal structure, in particular in bone mineral density. Nevertheless, the knowledge regarding the eff...

ba0001pp221 | Cell biology: osteoclasts and bone resorption | ECTS2013

Modulation of osteoclastogenesis by antihypertensive drugs

Oliveira Teresa , Costa-Rodrigues Joao , Ferraz Ricardo , Prudencio Cristina , Fernandes Maria

Despite its rigid structure, bone is a dynamic tissue that is in constant remodeling. This process requires the action of the bone-resorbing osteoclasts and the bone-synthesing osteoblasts. One of the adverse effects attributed to some antihypertensive agents is the ability to alter normal bone metabolism. However, their effective actions on human bone cells remain to be clarified. In this work, the effects of five calcium channel blockers, a class of antihypertensive drugs (A...