Searchable abstracts of presentations at key conferences on calcified tissues
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6th International Conference on Children's Bone Health

Rotterdam, The Netherlands
22 Jun 2013 - 25 Jun 2013

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6th International Conference on Children's Bone Health , 22 - 25 June 2013; Rotterdam, The Netherlands

Oral Communications


ba0002oc19 | Miscellaneous | ICCBH2013

New therapeutic approach in OI VI: suppression of bone resorption using the RANKL antibody denosumab

Hoyer-Kuhn Heike , Semler Oliver , Netzer Christian , Dotsch Jorg , Schonau Eckhard

Background: Osteogenesis imperfecta (OI) as a rare disease is characterized by reduced bone mass, increased fracture rate, bone deformities and skeletal pain.Currently patients are treated with i.v. bisphosphonates regardless of the underlying mutation.Recently the gene causing OI type VI was described (SERPINF-1, altered RANKL-pathway). This leads to a new understanding of the underlying pathophysiology and offered a new therapeut...

ba0002oc20 | Miscellaneous | ICCBH2013

Exercise completed when young provides lifelong benefit to cortical bone structure and estimated strength

Warden Stuart , Roosa Sara Mantila , Hurd Andrea , Fuchs Robyn

Objectives and methods: Exercise induces greatest bone gains during growth, yet reduced bone strength is an age-related phenomenon. This raises the question of whether exercise-induced bone changes when young persist into adulthood. Previous work has suggested exercise-induced gains in bone mass are lost with aging. However, exercise during growth primarily influences bone structure rather than mass to increase strength and mechanisms exist for the maintenance of exercise effe...

ba0002oc21 | Miscellaneous | ICCBH2013

A randomized, double-blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone

Boyce Alison M , Kelly Marilyn H , Brillante Beth A , Kushner Harvey , Wientroub Shlomo , Riminucci Mara , Bianco Paolo , Robey Pamela G , Collins Michael T

Fibrous dysplasia (FD) is a benign skeletal disease caused by activating mutations of Gsα. These mutations lead to formation of abnormal and mechanically unsound bone and fibrotic tissue. Clinical sequelae include deformity, fracture, and pain. Studies in bisphosphonates have shown improvement in bone pain and inconsistent effects on FD mineralization; however interpretation has been limited by a lack of controlled trials.Objecti...

ba0002oc22 | Miscellaneous | ICCBH2013

Prolidase deficient mice are osteoporotic in early life

Foster Sarah , Grabowski Peter , Gallagher Orla , Besio Roberta , Rossi Antonio , Bishop Nick , Forlino Antonella

Background: Proline and hydroxyproline account for ~25% of aminoacids in collagen., Prolidase (peptidase D (EC, cleaves iminodipeptides with a C-terminal proline or hydroxyproline, playing a major role in collagen catabolism. Mice with prolidase deficiency (PD) present with varied phenotypes including reduced size compared to wild-type littermates. We measured structural and mechanical properties of bones in PD mice.Methods: Whole femurs from ...

ba0002oc23 | Miscellaneous | ICCBH2013

Switching from alendronate to RANKL blockade alters bone properties after 14 weeks of therapy in the oim/oim mouse

Marino Josephine , Pleshko Nancy , Doty Steve , Carter Erin , Boskey Adele , Raggio Cathleen

Objective: The purpose of this study was to determine whether osteogenesis imperfecta (OI) patients entering adulthood should continue with bisphosphonate therapy or would benefit from switching to a RANKL blockade therapy. To address this question, we used a mouse model of type III OI.Methods: Animal studies were performed under IACUC approval. OI (oim/oim) and wild-type (WT) mice were treated from 2–26 weeks with i) saline; ii) alendronat...

ba0002oc24 | Miscellaneous | ICCBH2013

Fibroblast Growth Factor 23 Plasma Levels Are Elevated with Early Chronic Kidney Disease and Positively Associated with Steroid Based vs Steroid Free Immunosuppression in Pediatric Renal Transplant Patients

Srivastava Rachana , Srivaths Poyyapakkam , Brewer Eillen

Background: Fibroblast growth factor 23 (FGF23) is a circulating phosphaturic hormone that also suppresses renal 1α-hydroxylase activity. In adult patients (pts) plasma FGF23 increases in early stages of chronic kidney disease (CKD), even before serum phosphorus changes. In a study of 984 adult renal transplant pts, higher FGF23 levels were independently associated with increased risk of all-cause mortality and allograft loss during 39 months follow-up2</sup...