Searchable abstracts of presentations at key conferences on calcified tissues

ba0002p65 | (1) | ICCBH2013

Improvement in morphological properties of trabecular bones and longitudinal growth in tibia for growing rats through an impact stimulation

Eom Sinae , Ko Chang-Yong , Park Ji Hyung , Seo Dong-Hyun , Jung Young Jin , Kim Han Sung

Mechanical stress and strain generated by physical exercises or the other passive stimulations are well known to have a positive effect on the growing musculoskeletal system. Especially, when the impact stimulation which evokes high magnitude of strain in a second is applied to bone, it improves bone qualities. Thus, to verify the effect of impact stimulation, we conducted longitudinal study on morphological properties of the tibia in growing rats. Free falls from designated h...

ba0002p155 | (1) | ICCBH2013

Assessment of bone density in MPS IV (Morquio disease)

Kecskemethy Heidi , Harcke H Theodore , Ruhnke Kristen , Tomatsu Shunji

Objectives: i) Describe bone mineral density (BMD) of children with MPS IV (Morquio disease), a rare genetic disorder which produces skeletal deformity, small stature and results in physical limitations such as the ability to walk.ii) Examine fracture history and factors affecting bone health in Morquio.iii) Describe technical issues encountered in assessing BMD by DXA in Morquio.Methods: In this prospective ...

ba0002p157 | (1) | ICCBH2013

Growth and bone health after hematopoietic stem cell transplantation or tyrosine kinase inhibitors in children with chronic myeloid leukemia: a single institution experience

Dilley Kimberley , Broglie Larisa , Chaudhury Sonali , Hijiya Nobuko

Objective: To examine the impact on growth and bone mineral density (BMD) of tyrosine kinase inhibitors (TKI) vs hematopoeitic cell transplant (HCT) for treatment of chronic myelogenous leukemia (CML) in patients <18 years of age.Methods: We performed a retrospective review of children with CML in chronic phase treated between 1992 and 2011 at a single institution and evaluated available growth and BMD data.Results: Twenty-five...

ba0002p196 | (1) | ICCBH2013

Bigger but not stronger? GH treatment in Turner syndrome may confer no benefit to HR-pQCT determined bone micro-architecture

Nour Munier , Boyd Steven K , Perry Rebecca J , Stephure David K , Hanley David A

Turner syndrome (TS) is known to be associated with increased risk of osteoporosis and fracture. Childhood treatment with GH has been considered standard of care for treatment of growth failure in TS, while the influence of GH on bone health has been poorly understood. The purpose of this study is to assess the influence of GH on bone microarchitecture on a cohort of TS subjects.TS subjects aged 16–45 were included. Bone mineral density (BMD) was as...

ba0002lb2 | (1) | ICCBH2013

WNT1 mutations in early-onset osteoporosis and osteogenesis imperfecta identify a key WNT ligand regulating bone mass

Laine Christine , Joeng Kyu Sang , Campeau Philippe , Kiviranta Riku , Tarkkonen Kati , Grover Monica , Lu James , Pekkinen Minna , Wessman Maija , Heino Terhi , Nieminen-Pihala Vappu , Laine Tero , Kroger Heikki , Cole William , Lehesjoki Anna-Elina , Krakow Deborah , Curry Cynthia , Cohn Daniel , Gibbs Richard , Lee Brendan , Makitie Outi

The role of the WNT pathway in skeletal maintenance has been extensively studied since the identification of mutations in key signaling WNT mediators (LRP5 and sclerostin) in high and low bone mass phenotypes. However, the identity of the key WNT ligand that signals via LRP5/6 has remained unknown. We aimed to identify genes with a major effect on the skeleton by studying individuals and families with early-onset osteoporosis or osteogenesis imperfecta (OI).<p class="abste...

ba0003pp236 | Osteoporosis: evaluation and imaging | ECTS2014

Improved assessment of vertebral cortex thickness by means of analytical deconvolution of radial bone mineral density distributions

Damm Timo , Pena Jaime , Bastgen Jan , Krause Matthias , Campbell Graeme , Barkmann Reinhard , Gluer Claus-Christian

New treatment agents against osteoporosis may not only lead to an improved trabecular structure, but can probably also strengthen the cortex. To assess this treatment effects by means of QCT, one has to deal with significant partial volume effects observing this very thin, but compact structure. A new method for cortical thickness estimation has been developed using an analytical deconvolution approach. After estimating the point-spread-function (PSF) of a scanner/kernel combi...

ba0003pp332 | Osteoporosis: treatment | ECTS2014

Denosumab therapy of osteoporosis in patients after solid organ transplantation and impaired renal function

Brunova Jana , Kratochvilova Simona

The therapy of osteoporosis in patients with end organ failure and after the transplantation (Tx) becomes more frequent problem with the better patients and graft survival rates. Moreover some of these patients have impaired renal function and therefore cannot use bisphosphonates. The supplementation therapy with calcium and/or vitamin D is frequently not sufficient to improve bone mass. The possible innovative therapy is Denosumab (Prolia inj.) - MAB to RANK, which is not con...

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...

ba0004op9 | (1) | ICCBH2015

Growth, body mass index, bone health and ambulatory status of boys with Duchenne Muscular Dystrophy treated with daily vs intermittent oral glucocorticoid regimen

Crabtree Nicola , Padidela Raja , Shaw Nicholas , Hogler Wolfgang , Roper Helen , Hughes Imelda , Adams Judith , Daniel Anjali , Mughal Zulf

Background: Oral glucocorticoids (GC; prednisolone dose of 0.75 mg/kg per day), help to preserve muscle strength and prolong independent walking in boys with DMD. This study compared longitudinal growth, body mass index (BMI), bone mineral density (BMD), vertebral fractures (VFs) and ambulatory status in boys with DMD on daily (DAILY) or intermittent (INTER; 10 days on & 10 days off), oral GC regimens.Methods: 50 DMD boys from two UK centres were inc...

ba0004p2 | (1) | ICCBH2015

The assessment of bone regulatory pathways in children with malignant bone tumors

Ambroszkiewicz Jadwiga , Gajewska Joanna , Chelchowska Magdalena , Rogowska Elzbieta , Rychlowska-Pruszynska Magdalena

Objectives: As the receptor activator of the nuclear factor κB (RANK)/receptor activator of the nuclear factor κB ligand (RANKL)/osteoprotegerin (OPG) cytokine system is essential for osteoclastogenesis and Wnt signaling pathway for osteoblastogenesis, we decided to assess these two main bone regulatory pathways in patients with malignant bone tumors on the completion of anticancer therapy.Methods: The study included 35 patients (median age 15....