Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p145 | (1) | ICCBH2019

Nine-month follow-up data on biochemical, clinical, radiological and functional parameters in a clinical cohort of children at Evelina London Children's Hospital with X-linked hypophosphataemia treated with Burosumab

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

Burosumab, a monoclonal antibody targeting fibroblast growth factor 23, is now available for clinical use in children with X-linked hypophosphatemia (XLH). We aimed to explore the effects of burosumab in children with XLH in a clinical setting, considering: a) Biochemistry b) Growth c) Lower limb deformity (LLD) d) Radiology e) Motor function<p class="abstext"...

ba0004p81 | (1) | ICCBH2015

Liver growth hormone receptor signaling in chronic kidney disease related growth retardation: the role of suppressor of cytokine signaling 2

Hilal Rawan Abu , Assadi Mohammad Hani , Chen Yu , Medrano Juan , Rabkin Ralph , Segev Yael , Landau Daniel

Introduction: Children with chronic kidney disease (CKD) who suffer from growth retardation have usually normal circulating GH but low IGF1, suggesting GH resistance. We have previously shown in growth retarded CKD rats an increase in suppressor of cytokine signaling 2 (SOCS2) levels, a key negative regulator of GH signaling. SOCS2 spontaneously mutated (HG) mice show an exaggerated body growth and increased bone mass. We investigated growth and GH receptor (GHR) signaling in ...

ba0002op11 | (1) | ICCBH2013

Artistic gymnasts display micro-architectural advantages over trampolining and tumbling gymnasts: a high resolution peripheral quantitative computed tomography study

Burt Lauren A , Stanger Scott P , Schipilow John D , Boyd Steven K

Participation in high impact sports during growth increases bone quality. Gymnasts have previously displayed increased bone mass and strength at both the upper and lower limbs compared with controls. However, it is not yet understood how bone microarchitecture is affected by gymnastics participation, and if this differs based on gymnastics discipline. Therefore, the objective of this study was to investigate the influence of gymnastics discipline on bone microarchitecture in a...

ba0001pp275 | Genetics | ECTS2013

Genetic aspects of bone remodeling disturbance in patients with aggressive periodontitis

Zinovyeva Anastasia , Atrushkevich Victoria , Polyakov Alexander

Introduction: Aggressive periodontitis (AgP) is an inflammatory disease causing rapid loss of teeth in young patients.Aim: Determine degree of impact of COL1A1 gene on likelihood of AgP development.Materials and methods: Study included 47 patients with AgP, 40 patients with osteoporosis (OP), and 64 healthy patients (HP). Polymorphic variant c.104-441G>T (COL1A1) was studied. Statistics: Fisher’s...

ba0005ni6 | Abstract Presentations | ECTS2016

Multi-potency and immunosuppressive activity of mesenchymal stromal cells derived from human induced pluripotent stem cells

Roux Clemence , Saviane Gaelle , Pini Jonathan , Dihib Gihen , Nourhene Belhaid , Wakkach Abdel , Blin-Wakkach Claudine , Rouleau Matthieu

Tissue healing/reconstruction as well as exacerbated inflammatory diseases may benefit from stem cell based therapies. Ex vivo isolated tissue mesenchymal stromal cells (MSCs) displaying multi-potent activity and immune-regulatory functions were long ago proposed as therapeutic cells and already tested in many clinical assays. Nevertheless, their use may be restricted because of the few number that can be recovered from adult tissues, their limited in vitro e...

ba0005p80 | Bone Marrow | ECTS2016

Multi-potency and immunosuppressive activity of mesenchymal stromal cells derived from human induced pluripotent stem cells

Roux Clemence , Saviane Gaelle , Pini Jonathan , Dihib Gihen , Nourhene Belhaid , Wakkach Abdel , Blin-Wakkach Claudine , Rouleau Matthieu

Tissue healing/reconstruction as well as exacerbated inflammatory diseases may benefit from stem cell based therapies. Ex vivo isolated tissue mesenchymal stromal cells (MSCs) displaying multi-potent activity and immune-regulatory functions were long ago proposed as therapeutic cells and already tested in many clinical assays. Nevertheless, their use may be restricted because of the few number that can be recovered from adult tissues, their limited in vitro e...

ba0003pp312 | Osteoporosis: treatment | ECTS2014

Farnesylpyrophosphate synthase rs2297480 polymorphism and the response to the zoledronic acid in the treatment of postmenopausal osteoporosis

Yureneva Svetlana , Yakushevskaya Oksana , Donnikov Andrey , Kuznetzov Sergey , Mullambaeva Svetlana , Alekseeva Marina , Trophymov Dmitry , Ivanetz Tatyana , Sukhikh Gennady

Research design and methods: A characterisation of 225 European (Russian) osteoporotic postmenopausal women, treated for 2 years with amino-bisphosphonate zoledronic acid (zol), with respect to the adenosine/cytosine (A/C) rs2297480 farnesyl pyrophosphate synthase (FDPS) gene polymorphism, was carried out by PCR-based enzymatic digestion and quantitative PCR allelic discrimination on genomic DNA extracted from blood leukocytes. The association between these polymorphism genoty...

ba0007p46 | (1) | ICCBH2019

Vertebral fractures are more prevalent than long bone fractures in boys with glucocorticoid-treated Duchenne Muscular Dystrophy: Results of a prospective observational study

Jackowski Stefan A , McAdam Laura C , Ma Jinhui , McMillan Hugh J , Konji Victor N , Matzinger Mary Ann , Shenouda Nazih , Jaremko Jacob L , Koujok Khaldoun , Khan Nasrin , Khatchadourian Karine , Robinson Marie-Eve , MacLeay Lynn , Saleh David S , Siminoski Kerry , Rauch Frank , Ward Leanne M

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

ba0005lb3 | (1) | ECTS2016

Peripheral quantitative computed tomography measures contribute to the understanding of bone fragility in low-trauma fracture patients

Jiang Hongyuan , Yates Christopher , Gorelik Alexandra , Kale Ashwini , Song Qichun , Wark John D

Background and aims: Dual energy X-ray absorptiometry (DXA) as currently utilised has limitations in identifying patients with osteoporosis and predicting fractures, since most low-trauma fracture (LTF) patients have osteopenia not osteoporosis based on DXA assessment. We aimed to express peripheral quantitative computed tomography (pQCT) variables of patients with low-trauma fracture as T-scores by using T-score scales obtained from healthy young women, and ...

ba0004is26biog | (1) | ICCBH2015

Duchenne and cerebral palsy

Munns Craig

Biographical DetailsAssociate Professor Craig Munns is a Senior Staff Specialist in Bone and Mineral Medicine and Endocrinology at the Children’s Hospital at Westmead and Conjoint Associate Professor in the Sydney Medical School at the University of Sydney, Australia. Following the completion of his Paediatric and Endocrinology training at The Royal Children’s Hospital, Brisbane...