Searchable abstracts of presentations at key conferences on calcified tissues

ba0002oc14 | Diagnostics | ICCBH2013

What DXA measurement sites are best for bone health assessment in children? Effect of inter-machine differences on bone outcomes from the Bone Mineral Density in Childhood Study

Zemel Babette , Kalkwarf Heidi , Leonard Mary , Gilsanz Vicente , Lappe Joan , Shults Justine , Shepherd John , Oberfield Sharon , Winer Karen

Objective: Total body less head (TBLH) and spine are the recommended DXA sites for bone health assessment in children and adolescents. However, inter-machine differences will affect use and interpretation of results in clinical care and research applications. We examined BMC and areal-BMD (aBMD) at 4 skeletal sites among healthy children to identify the magnitude of inter-machine differences in Z-scores.Methods: BMDCS evaluated healthy participa...

ba0002oc29 | Chronic diseases | ICCBH2013

Mineral metabolism, cortical volumetric bone mineral density and fracture risk in childhood chronic kidney disease

Denburg Michelle , Tsampalieros Anne , de Boer Ian , Shults Justine , Kalkwarf Heidi , Zemel Babette , Foerster Debbie , Stokes David , Leonard Mary

Background and Objectives: The relations among cortical volumetric bone mineral density (CortBMD) and comprehensive measures of mineral metabolism have not been addressed in chronic kidney disease (CKD). The aim was to identify determinants of CortBMD in childhood CKD. A secondary objective was to assess if CortBMD was associated with subsequent fracture.Design/participants: This prospective cohort study in children, adolescents and young adults (ages 5&...

ba0002p156 | (1) | ICCBH2013

Reflection analysis of infant scans results may improve infant DXA bone density and body composition result that contain motion

Shepherd John , Fan Bo , Powers Cassidy , Stranix-Chibanda Lynda , Fowler Mary , DiMeglio Linda , George Kathy , Siberry George

Objectives: Special dual-energy X-ray absorptiometry (DXA) protocols permit quantification of bone mineralization, fat mass, and fat distribution in infants. Our objective was to evaluate the accuracy and precision of a multiscan acquisition protocol designed to allow for reflection and imputation analysis for regions with movement.Methods: The IMPAACT P1084s Study assesses bone and kidney safety of antiretrovirals used for PMTCT. Newborns received a spi...

ba0002oc1 | Epidemiology | ICCBH2013

The Amalgamated Paediatric Bone Density Study (The ALPHABET Study): the collation and generation of UK based reference data for paediatric bone densitometry

Crabtree Nicola , Machin Mike , Bebbington Natalie , Adams Judith , Ahmed Faisal , Arundel Paul , Bishop Nicholas , Fewtrell Mary , Hogler Wolgang , Mughal M Zulf , Rhodes Laura , Shaw Nicholas , Ward Kate

Understanding normal patterns of bone growth is important for optimising bone health in children and reducing osteoporotic fractures in later life. Recently published guidelines for bone assessment in children state that to predict fractures a technique should identify children at risk of clinically significant fractures and that dual-energy absorptiometry (DXA) is the preferred method of assessment. Despite these guidelines there is still inconsistency and lack of consensus r...

ba0003pp90 | Bone development/growth and fracture repair | ECTS2014

A randomized, double-blind, placebo-controlled, ascending, single-dose study of a human monoclonal anti-FGF23 antibody (KRN23) in X-linked hypophosphatemia

Carpenter Thomas , Imel Erik , Ruppe Mary , Weber Thomas , Klausner Mark , Wooddell Margaret , Kawakami Tetsuyoshi , Ito Takahiro , Zhang Xiaoping , Humphrey Jeffrey , Insogna Karl , Peacock Munro

Purpose: In X-linked hypophosphatemia (XLH), elevated serum FGF23 causes low serum phosphorus (Pi) and inappropriately normal 1,25-dihydroxyvitamin D (1,25(OH)2D) levels. We report safety, tolerability and biochemistry markers following single ascending dose administration of KRN23 in adults with XLH.Methods: 38 XLH subjects with baseline FGF23≥30 pg/ml were randomized to receive single doses of KRN23 (K) or placebo (P) either (0.003&#15...

ba0003pp91 | Bone development/growth and fracture repair | ECTS2014

Pharmacokinetics and pharmacodynamics of a human monoclonal anti-FGF23 antibody (KRN23) after ascending single-dose administration in patients with X-linked hypophosphatemia

Zhang Xiaoping , Carpenter Thomas , Imel Erik , Ruppe Mary , Weber Thomas , Klausner Mark , Kawakami Tetsuyoshi , Ito Takahiro , Humphrey Jeffrey , Insogna Karl , Peacock Munro

Purpose: In X-linked hypophosphatemia (XLH), elevated serum FGF23 causes low serum phosphorus (Pi) and inappropriately normal 125-dihydroxyvitamin D (125(OH)2D) levels. We report PK and PD of KRN23 following single ascending dose administration in adults with XLH.Methods: 38 XLH patients with baseline FGF23≥30 pg/ml were randomized to receive a single dose of KRN23 (K) or placebo (P) either i.v. (0.003–0.3 mg/kg) or s.c. (0.1–1...

ba0006oc20 | (1) | ICCBH2017

Autoimmune hyperphosphatemic tumoral calcinosis

Ramnitz Mary Scott , Burbelo Peter , Egli-Spichtig Daniela , Perwad Farzana , Romero Christopher , Ichikawa Shoji , Farrow Emily , Econs Michael , Guthrie Lori , Gafni Rachel I. , Collins Michael T.

Background: Hyperphosphatemic familial tumoral calcinosis (HFTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is an autosomal recessive disorder due to deficiency of or resistance to intact fibroblast growth factor 23 (FGF23). This leads to hyperphosphatemia, increased renal reabsorption of phosphorus (TRP), and elevated or inappropriately normal 1,25-dihydroxyvitamin D (1,25D). Affected individuals may develop ectopic calcifications and/or diaphyseal hyperostosis. Mutations ...

ba0007oc7 | (1) | ICCBH2019

Comparison of zoledronate and pamidronate in children with skeletal disorders: Short term safety experience from a single institution

Tosi Laura , Estrada Andrea , Floor Marianne , Kim Mirini , Weigley Lindsay , Dollar Christina , Gillies Austin , Roberts Mary Scott , Gafni Rachel , Boyce Alison

Objectives: Bisphosphonates are frequently used in children with skeletal disorders, however optimal dosing and regimens are unknown. Early treatment focused on pamidronate (PAM), a second-generation formulation, however use of zoledronate (ZOL), a more potent third-generation bisphosphonate, has recently increased due to shorter and less frequent infusions. The objective of this study is to compare short-term safety of ZOL and PAM in a pediatric population.<p class="abste...