Searchable abstracts of presentations at key conferences on calcified tissues

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...

ba0006p085 | (1) | ICCBH2017

Cumulative radiation exposure from diagnostic imaging and associated lifetime cancer risk in children with Osteogenesis Imperfecta

Thorby-Lister Amy , Hogler Wolfgang , Hodgson Kirsten , Crabtree Nicola , Shaw Nick , Saraff Vrinda

Background and objectives: Fracture rate in Osteogenesis Imperfecta (OI) is highest between 0 and 19 years, and associated radiation exposure also carries the highest lifetime cancer risk. Here, we investigate the cumulative effective radiation dose (E) and lifetime cancer risk from diagnostic imaging in OI children. We also explore the hypothesis that negative family history of OI will increase injury-related, fracture-negative X-rays due to parental anxiety.<p class="abs...

ba0001pp77 | Bone development/growth and fracture repair | ECTS2013

Histologic diagnosis of aluminum osteomalacia in renal failure rats

Teraki Yoshimi

Aim of the study: Animal studies have also been reported demonstrating enhanced development of osteomalacia and increased osteoids following long-term high-dose administration of Al compound in rats, especially in those with renal failure rats (Robertson et al.).For histologic detection of Al. aluminon and other staining reagents have usually been used but are not adequately sensitive and lack in specificity. We have recently developed a method ...

ba0001pp460 | Other diseases of bone and mineral metabolism | ECTS2013

Osteopontin ASARM peptide binding to crystal faces of hydroxyapatite – computational simulations

Mansouri Ahmad , Masica David , Gray Jeffrey , McKee Marc

ASARM peptide (acidic, serine- and aspartate-rich motif) and osteopontin (OPN) fragments accumulate in X-linked hypophosphatemia patients and/or in the Hyp mouse model and, when phosphorylated, potently inhibit mineralization in osteoblast cultures. To investigate this inhibition, we modeled the binding to hydroxyapatite of the human OPN-ASARM peptide (DDSHQSDESHHSDESDEL) using RosettaSurface computational simulations. Peptide binding to hydroxyapatite atomic planes constructe...

ba0001pp475 | Other diseases of bone and mineral metabolism | ECTS2013

Multidisciplinary studies of ancient calcified tissues: renal stones from mummies

Capulli Mattia , Arrizza Lorenzo , Rucci Nadia , Piperni Sara Gemini , Quaresima Raimondo , Giuffra Valentina , Fornaciari Gino , Teti Anna , Ventura Luca

The renal stones found in the mummies of Pandolfo III Malatesta, Lord of Fano (1370–1427) and an anonymous nobleman from Popoli (XVIII century) were investigated using different techniques. Both specimens were examined with binocular stereomicroscopy (BSM) and scanning electron microscopy (SEM), also with energy dispersive X-ray analysis (EDX). Multiple tiny fragments from surface and inner portions were submitted to X-ray diffraction (XRD) analysis. Subsequently, the cal...