Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p105 | (1) | ICCBH2015

Refractory hypercalcaemia of malignancy: responsiveness to Denosumab and Zoledronate

Giri Dinesh , Ramakrishnan Renuka , Hayden James , Brook Lynda , Das Urmi , Mughal M Zulf , Selby Peter , Dharmaraj Poonam , Senniappan Senthil

Background: Hypercalcaemia secondary to malignancy is rare in children and adolescents. Parathyroid hormone related peptide (PTH-rP) secreted by malignant cells increases bone resorption and renal calcium retention causing hypercalcaemia. We report two cases of hypercalcaemia of malignancy refractory to treatment with pamidronate and corticosteroids but responsive to treatment with Denosumab and Zoledronic acid.Case 1: Presenting problem: A 17-year-old b...

ba0004p199 | (1) | ICCBH2015

LRP5-associated high bone mass disorder: novel familial mutation in LRP5 and investigation of bone mineralization density distribution (BMDD)

Roetzer Katharina M , Uyanik Goekhan , Brehm Attila , Zwerina Jochen , Zandieh Shahin , Czech Thomas , Roschger Paul , Klaushofer Klaus

Mutations in LRP5 cause a variety of phenotypes, including high bone mass and low bone mass disorders. In patients with high bone mass, different heterozygous mutations have been described, all of them clustering in a region including the binding pocket for DKK1 and sclerostin. The pathogenic mechanism is thought to be a gain-of-function mediated by an impaired inhibition of the canonical Wnt signalling pathway, thereby leading to increased bone modelling.<p class="abstext...

ba0005p487 | Preclinical and ex vivo imaging | ECTS2016

The CAM assay for human bone regeneration evaluation: the potential of Laponite® clay gel for growth factor delivery ex vivo

Moreno-Jimenez Ines , Hulsart-Billstrom Gry , Lanham Stuart A , Kanczler Janos M , Evans Nicholas D , Oreffo Richard O C

An increasing number of biomaterials are in development, seeking to mimic the natural cascade of events during fracture repair. However, these biomaterials need to be rigorously tested prior to clinical application. In vitro testing lacks the physiological environment, while in vivo studies do not always predict the patient response. Here we hypothesize that the chorioallantoic membrane (CAM) assay can be used to culture human living bone and we aim to examin...

ba0006p038 | (1) | ICCBH2017

Hypophosphatasia associated with acute disseminated encephalomyelitis (ADEM): causal relationship or coincidence?

Jacobs Benjamin , Gall Angela , Peeva Daniela , Lacassagne Sandrine , Talwar Dinesh , Wakeling Emma L , Tenorio Jair , Mughal M Zulf

Background: Hypophosphatasia is generally regarded as a disease of bone and teeth. Lack of Tissue Non-Specific Alkaline Phosphatase (TNAP) leads to an accumulation of inorganic pyrophosphate and the Vitamin B6 metabolite pyridoxal 5′-phosphate (PLP), a reduction in pyridoxic acid (PA) and increased PLP/PA ratio. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters, and is a well-recognised cause of neonatal seizures. We have found no previous reports...

ba0006p145 | (1) | ICCBH2017

Longitudinal growth and bone development in glucocorticoid treated boys with Duchenne muscular dystrophy

Joseph S , Capaldi N , DiMarco M , Dunne J , Horrocks I , Shepherd S , Ahmed S F , Wong S C

Background: There is still limited information on changes in growth especially segmental growth and bone mass of glucocorticoid(GC) treated boys with Duchenne Muscular Dystrophy (DMD).Objectives: To evaluate changes in growth and bone mass in GC treated boys with DMD.Methods: Retrospective study of 15 boys with DMD treated with GC, median age 7.6 years (4.1, 15.5) who had repeated DXA scan for clinical monitoring of bone health, me...

ba0006lb19 | (1) | ICCBH2017

Selected risk factors of fractures in children -own observation

Jakubowska-Pietkiewicz Elżbieta , Fendler Wojciech , Młynarski Wojciech , Chlebna-Sokoł Danuta , Matusik Paweł

Bone fractures may depend on Vitamin D Receptor Gene (VDR), bone mineral density, bone turnover markers.Patients and methods: About 161 patients were recruited and underwent: skeletal densitometry (DXA) method and bone turnover studies (Osteocalcin and Ntx).The study group was evaluated using restriction enzyme digestion at BsmI (rs1544410), FokI (rs2228570), ApaI (rs7975232) and TaqI (rs731236), polymorphic sites of t...

ba0003pp162 | Cell biology: osteoclasts and bone resorption | ECTS2014

The role of IL-6 as a modulator of osteoclastic resorption in vitro in acute Charcot osteoarthropathy

Petrova Nina , Petrov Peter , Edmonds Michael , Shanahan Catherine

Aims: To investigate the role of interleukin-6 (IL-6) as a modulator of osteoclastic resorption in vitro in acute Charcot osteoarthropathy.Material and methods: Peripheral blood mononuclear cells were isolated from six patients with acute Charcot osteoarthropathy, five diabetic and five healthy controls and cultured in vitro on bovine bone disks for 21 days in the presence of i) macrophage-colony stimulating factor (M-CSF) and receptor ...

ba0005p321 | Osteoporosis: evaluation and imaging | ECTS2016

The T-score standard in osteoporotic sheep model: insight into independent reference group

Khassawna Thaqif El , Engelhardt Markus , Weisweiler David , Bocker Wolfgang , Schmitt Jakob , Heiss Christian

FDA guidelines recommend the sheep as large animal model for osteoporosis. Studies of osteoporosis induction use dual energy x-ray absorptiometry (DEXA) to evaluate bone status. Experimentally, the T-score is calculated based on reference bone mineral density (BMD) values of animals before treatment (0 M). Whereas clinically the reference BMD represents values of an independent group of patients around 30 years old. The study hypothesize that the use of additional ind...

ba0002oc28 | Chronic diseases | ICCBH2013

Bone mineral density at diagnosis determines fracture rate in children-treated according to the DCOG-ALL9 protocol

te Winkel Mariel Lizet , Pieters Rob , Hop Wim C J , Roos Jan C , van der Sluis Inge M , Bokkerink Jos P M , Leeuw Jan A , Bruin Marrie C A , Kollen Wouter J W , Veerman Anjo J P , de Groot-Kruseman Hester A , van den Heuvel-Eibrink Marry M

Objectives: To elucidate the incidence and risk factors of skeletal toxicity in children with ALL treated with the dexamethasone-based DCOG-ALL9 protocol.Methods: Prospectively, the cumulative incidence of fractures was assessed in 672 patients and compared between different subgroups using the log-rank test. Serial measurements of bone mineral density of the lumbar spine (BMDLS) were performed in 399 ALL patients using dual energy X-ray absor...