Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp183 | Cell biology: osteoblasts and bone formation | ECTS2013

Development of mice models to study implant osseointegration and failure in alveolar bone

Mouraret Sylvain , Bardet Claire , Hunter Dan J , Popelut Antoine , Brunski John B , Chaussain Catherine , Bouchard Philippe , Helms Jill A

Many of our assumptions concerning oral implant osseointegration are extrapolated from experimental models studying skeletal tissue repair in long bones rather than in oral bones. This discrepancy between clinical practice and experimental research hampers our understanding on how alveolar bone forms or resorbs around implants and how osseointegration of oral implants can be improved. To overcome this disconnect, we have developed a mouse model which mimics oral implant placem...

ba0003pp363 | Other diseases of bone and mineral metabolism | ECTS2014

Sustained efficacy and tolerability in infants and young children with life-threatening hypophosphatasia treated with asfotase alfa

Whyte Michael , Simmons Jill , Lutz Richard , Vallee Marc , Melian Agustin , Odrljin Tatjana , Bishop Nick

Background: Hypophosphatasia (HPP) results from inactivating mutation(s) in the gene for tissue non-specific alkaline phosphatase (TNSALP). Substantial improvement has been reported in skeletal mineralization and physical function in patients (pts) with life-threatening perinatal and infantile HPP treated for 48 weaks with asfotase alfa, a bone-targeted recombinant human TNSALP.Objective: To evaluate long-term efficacy and tolerability of asfotase alfa i...

ba0006p198 | (1) | ICCBH2017

Hearing the patient's voice: a focus group listening to the child and parent experiences of living with rare bone diseases

Massey Jill , Phillips Katie , Cornish Michael , Lawson Caron , Irving Melita , Cheung Moira

Objectives: • To establish the child and family experience of attending multi-disciplinary clinics within the rare bone disease service at Evelina London Children’s Hospital.• To gain an understanding of the daily challenges the children, young people and families face.• To understand how the tertiary multi-disciplinary team may support the child, young person and family.<p class="ab...

ba0002p62 | (1) | ICCBH2013

The effect of the ketogenic diet on the developing skeleton

Simm Peter , Bicknell-Royle Jill , Nation Judy , Draffin Kellie , Stewart Karen , Cameron Fergus , Scheffer Ingrid , Mackay Mark

Objectives: The ketogenic diet (KD) is a medically supervised, high fat, low carbohydrate and restricted protein diet which has been used successfully in patients with refractory epilepsy. Only one published report has explored its effect on the skeleton. We postulated that KD impairs bone mass accrual and examined skeletal health parameters in this patient group.Methods: Patients commenced on the KD from 2002–2009 were enrolled in a prospective, lo...

ba0007p74 | (1) | ICCBH2019

Burosumab can improve pain and quality of life for children with X-linked hypophosphataemia and their families: a London centre's experience

Gilbey-Cross Robyn , Sandy Jessica L , Morris Mavali , Cocca Alessandra , Sakka Sophia D , Massey Jill , Cheung Moira S

Objectives: Burosumab, a monoclonal antibody that therapeutically targets the underlying elevated levels of fibroblast growth factor 23 (FGF23) in X-linked hypophosphatemia (XLH), is now available to children out of trial conditions. Our objective was to describe the effect of burosumab on quality of life, functionality and pain in a clinical setting.Methods: Questionnaire tools were completed at baseline, 6 and 9 months for 9 children with XLH starting ...

ba0007p192 | (1) | ICCBH2019

Developing a high chair to meet the needs of infants with Achondroplasia; a collaboration between Evelina London Children's Hospital and Brunel University

Massey Jill , Phillips Kathryn , Lawrence Jack , Davies Angharad , Harris Laura , Cocca Alessandra , Spinelli Gabriella , Garaj Vanja , Irving Melita , Cheung Moira

Achondroplasia is the commonest form of dwarfism. Key features in infancy impacting positioning are rhizomelia, spinal deformities and large head size. Both standard high chairs and specialized adaptive seating are currently inadequate to meet the needs of these infants. This is due to their anatomical limitations and the guidance for supportive positioning in this group of children, which recommend that a flat, elongated spine is optimal, avoiding a ‘C shape’, as is...

ba0007oc14 | (1) | ICCBH2019

Burosumab resulted in greater improvement in clinical outcomes than continuation with conventional therapy in younger (1-4 years-old) and older (5-12 years-old) children with X-linked hypophosphatemia

Ward Leanne , Imel Erik , Whyte Michael , Munns Craig , Portale Anthony , Hogler Wolfgang , Simmons Jill , Padidela Raja , Namba Noriyuki , Cheong Hae , Nilsson Ola , Mao Meng , Skrinar Alison , Chen Chao-Yin , Martin Javier San , Glorieux Francis

Objective: We compared the efficacy and safety of burosumab, a monoclonal antibody against FGF23, to conventional therapy [oral phosphate and active vitamin D (Pi/D)] in children with X-linked hypophosphatemia (XLH).Methods: In this Phase 3 trial (NCT02915705), 61 children with XLH (1-12 years-old) were randomized 1:1 after a 7-day Pi/D washout to receive burosumab starting at 0.8 mg/kg SC Q2W or reinitiate Pi/D optimally titrated by investigators. Eligi...

ba0006pl1 | (1) | ICCBH2017

Poster List

Poster No. Category Title Author P002 Bone development A 3-year longitudinal study of skeletal effects and growth in children after kidney transplantation <a href="ba...