Searchable abstracts of presentations at key conferences on calcified tissues

ba0006p068 | (1) | ICCBH2017

Craniosynostosis can occur in children with nutritional rickets

Forestier-Zhang L , Arundel P , Cross R Gilbey , Mughal M Z , Offiah A C , Cheung M S

Background: Severe vitamin D deficiency (VDD), is a common disorder, which has complications including rickets, hypocalcaemia, hypotonia, delayed development and cardiomyopathy. Although nutritional rickets associated craniosynostosis has been reported, there is little awareness of this or knowledge about its clinical course or severity. We present five cases of late onset craniosynostosis in association with nutritional rickets.Clinical presentation: Th...

ba0006p090 | (1) | ICCBH2017

Intra-observer precision of vertebral height measurements using spine X-Rays And DXA in boys with Duchenne Muscular Dystrophy

Morrice R , Joseph S , Horrocks I , Shepherd S , Ahmed SF , Wong SC

Background: The role of untrained observers in evaluating vertebrae height and therefore detection of vertebral fracture (VF) from spinal radiographs (SXR) and dual energy-absorptiometry (DXA) images in children with concerns about osteoporosis is currently unknown.Objective: To assess intra-observer agreement of morphometric measurements of vertebra height by an untrained observer using SXR and DXA in boys with Duchenne Muscular Dystrophy (DMD).<p c...

ba0006p130 | (1) | ICCBH2017

The impact of intravenous bisphosphonate on vertebral morphometry in children with secondary osteoporosis and vertebral fractures

Timmons JG , Morrice R , Joseph S , Shepherd S , Mason A , Ahmed SF , Wong SC

Background: Intravenous (IV) bisphosphonate (BP) is used for treatment of painful vertebral fractures (VF) in children with underlying chronic conditions. BP effect on vertebral height reshaping in this population is however poorly studied.Aims/Objectives: To evaluate the impact of IV BP on vertebral morphometry in children with VF and underlying chronic medical conditions with associated glucocorticoid (GC) therapy.Methods: Retros...

ba0007p226 | (1) | ICCBH2019

The experience of canakinumab in 2 patients with primary tumor (tumoral) calcinosis

Kostik M , Petukhova V , Pigareva T , Likhacheva T , Idrisova R , Snegireva L , Krasnogorskaya O , Suspisin E

Background: Primary tumoral calcinosis is an orphan disease. The data about the incidence of this disease, as well as clinical recommendations for treatment are not presented in the literature.Presenting problem: Two patients – 11.5 years old boy and 8 years old girl with primary tumoral calcinosis had multiple foci of the subcutaneous calcification, walking impossibility, wheel-chair condition, fatigue, high fever and equinus deformity of the left ...

ba0002p21 | (1) | ICCBH2013

Bone disease in children with geroderma osteodysplasticum: a 25-year experience from a single tertiary centre

Gopal-Kothandapani J S , Padidela R , Clayton-Smith J , Chandler K E , Adams J E , Freemont A J , Mughal M Z

Geroderma osteodysplasticum (GO) is a rare autosomal recessive connective tissue disorder characterised by progeria like facies, wrinkled lax skin, joint hypermobility, congenital dislocation of hips and propensity to fragility fractures. In the past 25 years, five patients (three females and two males) diagnosed with GO were referred to our Paediatric metabolic bone service for assessment and management of secondary bone problems. All five children were born to consanguineous...

ba0003pp74 | Bone development/growth and fracture repair | ECTS2014

Bone turnover and FGF-23 levels in vitamin D-deficient critically ill patients with and without acute kidney injury

Schnedl Christian , Bisping Egbert , Zajic Paul , Dimai Hans Peter , Wagner Doris , Pieber Thomas R , Fahrleitner-Pammer Astrid , Amrein Karin

Introduction: Elevated FGF-23 serum levels are induced by hyperphosphatemia and are linked to poor skeletal mineralization and adverse outcomes including vascular calcification and mortality. Recently, it was shown that FGF-23 levels are substantially elevated in acute kidney injury (AKI), and that higher levels in AKI are associated with a greater risk of adverse outcomes.Methods: In 25 vitamin D deficient (25(OH)D <20 ng/ml) critically ill adults w...

ba0003pp101 | Cancer and bone: basic, translational and clinical | ECTS2014

The effect of granulocyte colony-stimulating factor in adults undergoing autologous peripheral blood stem cell collection

Amrein Karin , Drexler Camilla , Oczko Susanna , Sareban Nazanin , Sipurzynski Sabine , Pieber Thomas R. , Lanzer Gerhard , Wagner Doris , Fahrleitner-Pammer Astrid

Introduction: Granulocyte colony-stimulating factor (G-CSF) is widely used to mobilize peripheral blood stem cells (PBSC) and enable PBSC collection by apheresis. Although bone pain is a common adverse event following G-CSF treatment, little is known on its effect on bone metabolism.Methods: Markers of bone turnover (OC, osteocalcin, β-CTx, bALP, C-terminal telopeptide of type I collagen, bone specific alkaline phosphatase, TRAP, tartrate resistant ...

ba0003pp327 | Osteoporosis: treatment | ECTS2014

Baseline data of the DEVIDE-study: DEnosumab vs Intravenous Ibandronate: a 2-year retrospective head to head real life study

Amrein Karin , Muschitz Christian , Wagner Doris , Pieber Thomas R , Resch Heinrich , Dimai Hans Peter , Fahrleitner-Pammer Astrid

Introduction: Effective treatment of postmenopausal osteoporosis (PMO) is frequently compromised by poor adherence to short-term (≤1-monthly) medications. Aim of this study is to investigate the effect of parenteral ibandronate (IBN) compared to denosumab in a cohort of IBN pretreated PMO patients.Methods: In a retrospective analysis, a total of 808 women were treated with quarterly 3 mg IBN injections for 27+3 months and were regularly mo...

ba0003lb2 | (1) | ECTS2014

Bisphosphonates for osteoporosis and risk of breast cancer: misleading results from observational studies

Cummings Steven R , Hue Trisha , Cauley Jane A , Bauer Doug C , Ensrud Kristine E , Barrett-Connor Elizabeth , Black Dennis M

Purpose: To test whether bisphosphonates for osteoporosis reduce the risk of breast cancer.Background: Observational studies and meta-analyses find that women taking bisphosphonates have about a 1/3rd reduction in risk of breast cancer as soon as 1 year of treatment. This is plausible because bisphosphonates have in vitro anti-tumor activity and high doses of zoledronate reduce recurrence in women with breast cancer. However, observational studi...

ba0004op6 | (1) | ICCBH2015

Genetic variation is involved in impairment of bone mineral density in long-term adult survivors of childhood cancer

den Hoed M A H , Pluijm S M F , Stolk L , Uiterlinden A G , Pieters R , van den Heuvel-Eibrink M M

Introduction: Despite similarities in upfront treatment, impairment of bone mineral density(BMD) varies in long-term adult survivors of childhood cancer (CCS). We studied for the first time whether genetic variation is involved in impairment of BMD in adult long-term CCS.Method: This cross-sectional single-center cohort study included 334 adult CCS (median follow-up time: 15.2 years (range 5.1–39.8); median age at follow-up: 26.1 years (range 18.1&#...