Searchable abstracts of presentations at key conferences on calcified tissues

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 ...

ba0003pp187 | Genetics | ECTS2014

A novel mutation in IFITM5, encoding BRIL, impairs osteoblast production of PEDF and causes atypical type VI osteogenesis imperfecta

Reich Adi , Farber Charles R , Barnes Aileen M , Becerra Patricia , Rauch Frank , Cabral Wayne A , Bae Alison , Glorieux Francis H , Clemens Thomas L , Marini Joan C

Osteogenesis imperfecta (OI) type V is caused by a unique dominant mutation (c.−14C>T) in IFITM5, which encodes BRIL, a transmembrane ifitm-like protein most strongly expressed in osteoblasts, while type VI OI is caused by recessive null mutations in SERPINF1, encoding pigment epithelium-derived factor (PEDF). We identified a 25-year-old woman with severe OI, whose dermal fibroblasts and cultured osteoblasts displayed minimal secretion of PEDF, but ...

ba0001pp449 | Osteoporosis: treatment | ECTS2013

Odanacatib treatment reduces remodeling- and stimulates modeling-based bone formation in adult OVX monkeys

Chen C , Shih M , Zheng H , Duong L

Odanacatib (ODN), a selective and reversible cathepsin K inhibitor was shown to histomorphometrically reduce trabecular (Tb) and intracortical (Ic) bone remodeling while preserving endocortical (Ec) and stimulating periosteal (Ps) bone formation (BF) in monkeys. Here, we investigate the bone site specific mechanism of ODN on bone modeling (Mo) versus remodeling (Re)-based osteons. Rhesus monkeys (13–19 yrs, n=8–11/group) were ovariectomized and treated with ...

ba0005oc5.3 | Risk factors for fracture, Pagets disease of bone and musle and bone | ECTS2016

Osteoprotegerin autoantibodies are independently associated with low hip bone mineral density and increased fracture risk in patients with ankylosing spondylitis

Hauser Barbara , Zhao Sizheng , Visconti Micaela R , Riches Philip L , Goodson Nicola J , Ralston Stuart H

Osteoporosis and vertebral fractures are recognised complications of ankylosing spondylitis (AS) but the underlying causes are incompletely understood. Osteoprotegerin (OPG) is a decoy receptor for RANK-L and inhibits osteoclastogenesis. We have previously demonstrated that antibodies to OPG (OPG-Ab) are associated with osteoporosis and increased bone turnover in patients with autoimmune diseases. The aim of this study was to determine whether OPG-Abs were detectable in AS pat...

ba0003oc2.2 | Osteoporosis epidemiology | ECTS2014

Effect of daily vitamin B12 and folic acid supplementation on fracture incidence in elderly with an elevated plasma homocysteine level: B-PROOF, a randomized controlled trial

van Wijngaarden JP , Swart KMA , Enneman AW , Dhonukshe-Rutten RAM , van Dijk SC , Ham AC , Brouwer-Brolsma EM , van der Zwaluw NL , Sohl E , van Meurs JBJ , Zillikens MC , van Schoor NM , van der Velde N , Brug J , Uitterlinden AG , Lips P , de Groot CPGM

Background: Elevated plasma homocysteine levels are a risk factor for osteoporotic fractures. Supplementation with vitamin B12/folic acid lowers homocysteine levels. This study aimed to determine whether vitamin B12/folic acid supplementation reduces osteoporotic fracture incidence in hyperhomocysteinemic elderly.Methods: B-PROOF is a double-blind, randomized, placebo-controlled trial including 2 919 participants aged ≧65 years with elevated homocyste...

ba0003pp92 | Bone development/growth and fracture repair | ECTS2014

Effect of subcutaneous recombinant human parathyroid hormone, rhPTH(1--84), on skeletal dynamics in hypoparathyroidism: findings from the 24-week replace and 8-week relay phase III clinical trials

Bilezikian John P , Maruani Gerard , Rothman Jeffrey , Clarke Bart L , Mannstadt Michael , Vokes Tamara , Lagast Hjalmar , Shoback Dolores M

Hypoparathyroidism results in low bone turnover and increased bone mineral density (BMD). Replacing deficient PTH with rhPTH(1–84) has the potential to correct these skeletal abnormalities. To investigate the effect of rhPTH(1–84) on BMD and bone turnover markers (BTMs), data from two studies were assessed.REPLACE, a double-blind, multicenter, placebo-controlled study, randomized 134 patients with hypoparathyroidism to receive once-daily rhPTH(...

ba0002p145 | (1) | ICCBH2013

The recurrent IFITM5 c.−14C>T transition which causes osteogenesis imperfecta type V occurs at a highly methylated CpG dinucleotide: a novel mutational hot-spot?

Monti Elena , Mottes Margherita , Venturi Giacomo , Corradi Massimiliano , Gandini Alberto , Maines Evelina , Morandi Grazia , Piona Claudia , Antoniazzi Franco

Background: Osteogenesis imperfecta (OI) is a heterogeneous group of disorders characterized by bone fragility. The current classification comprises five forms (OI types I–V) with autosomal dominant inheritance and seven rarer forms (OI types VI–XII) with recessive inheritance. OI type V (MIM 610967) has distinguishing radiological features, such as propensity to hyperplastic callus formation, calcification of the forearm interosseous membrane, radial-head dislocatio...

ba0004p145 | (1) | ICCBH2015

Zoledronate as first line therapy for pediatric osteogenesis imperfecta?

Keemink Yvette , van Brussel Marco , Sakkers Ralph , Pruijs Hans , van Dijk Atty

Background: In pediatric osteogenesis imperfecta (OI), bisphosphonates (BPs) are considered the best treatment option to increase bone density and reduce fracture rate. Pamidronate (PAM) is regarded as standard care for moderate to severe OI. The most recent added BP is intravenous zoledronate (ZOL). ZOL has practical advances over PAM, however research on its efficacy and safety is scarce.Objectives: To investigate the efficacy and safety of treatment w...

ba0007p150 | (1) | ICCBH2019

Osteogenesis imperfecta: skeletal outcomes after bisphosphonates discontinuation at final height

Robinson Marie-Eve , Trejo Pamela , Palomo Telma , Glorieux Francis H , Rauch Frank

Objectives: Intravenous cyclical bisphosphonates are widely used to treat children with moderate to severe osteogenesis imperfecta (OI). They increase bone mineral density (BMD), diminish fracture rates and improve mobility. Bisphosphonates are often discontinued when growth is completed. We aimed to determine if the skeletal gains achieved with bisphosphonates persist after their discontinuation in patients with OI.Methods: We assessed patients with OI ...

ba0001pp364 | Osteoporosis: pathophysiology and epidemiology | ECTS2013

Osteoporosis an independent predictor of mortality in hip fracture patients

Diamantopoulos Andreas P , Hoff Mari , Hochberg Marc , Haugeberg Glenn

Introduction: Mortality after hip fracture is increased. However, only a few studies have explored for predictors beyond gender and age. Thus our aim was to study risk factors associated with increased mortality in hip fracture patients.Methods: Hip fracture patients (>50 years) admitted to a county hospital in 2004–2005 were consecutively invited for assessment at the hospital osteoporosis centre. A broad spectre of data was collected. Standard...