Searchable abstracts of presentations at key conferences on calcified tissues

ba0007oc3 | (1) | ICCBH2019

Osteocyte lacunae characteristics in healthy children

Blouin Stephane , Hartmann Markus A , Klaushofer Klaus , Glorieux Francis H. , Rauch Frank , Zwerina Jochen , Roschger Paul

Objectives: Osteocytes play a major role in bone metabolism as mechanosensors, key regulators of osteoblast and osteoclast activity and of the mineral homeostasis. Therefore the assessment of osteocytes characteristics is important to understand bone pathology. We propose to study indirectly the osteocytes by performing quantitative backscattered electron imaging to quantify the sectioned osteocyte lacunae density and size in 2D on bone samples.Methods: ...

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 ...

ba0007p118 | (1) | ICCBH2019

Methyl-CpG-binding protein 2 (MECP2) mutation type is associated with bone disease severity in Rett syndrome

Caffarelli Carla , Dea Tomai Pitinca Maria , Francolini Valentina , Canitano Roberto , De felice Claudio , Hayek Joussef , Gonnelli Stefano

Objective: Rett syndrome (RTT) is an X-linked neurodevelopment disorder. More than 95% of RTT female have mutations in methyl-CpG-binding protein 2 (MECP2), whose protein product modulates gene transcription. Specific MECP2 mutations may lead phenotypic variability and different degrees of disease severity. It is known that low bone mass is a frequent complication of subjects with Rett syndrome. This study aimed to investigate if specific MECP2 mutations may affects the degree...

ba0003pp292 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

CXCL8 and CCL20 enhance osteoblast-mediated osteoclastogenesis

Pathak Janak L , Bakker Astrid D , Verschueren Patrick , Lems Willem F , Luyten Frank P , Klein-Nulend Jenneke , Bravenboer Nathalie

Osteoporosis is common in rheumatoid arthritis (RA). Since osteoblasts express receptors for CXCL8 and CCL20, which are produced by inflammatory cells around the inflamed joints in RA, we hypothesized that CXCL8 and CCL20 contribute to osteoporosis in RA by affecting osteoblast proliferation, differentiation, and osteoblast-osteoclast communication.Primary human osteoblasts were cultured±CXCL8 (2–200 pg/ml) and CCL20 (5–500 pg/ml) for 14 d...

ba0005p43 | Bone development/growth and fracture repair | ECTS2016

Serum of patients with active rheumatoid arthritis inhibits differentiation of osteochondrogenic precursor cells

Pathak Janak L , Verschueren Patrick , Lems Willem F , Bravenboer Nathalie , Klein-Nulend Jenneke , Bakker Astrid D , Luyten Frank P

Delayed fracture healing is frequently experienced in patients with systemic inflammation such as during rheumatoid arthritis (RA). The reasons for this are diverse, but could also be caused by inflammatory cytokines and/or growth factors in serum from patients with active disease. We hypothesized that serum from patients with active RA contains circulating inflammatory factors that inhibit differentiation of osteochondrogenic precursors.Serum was obtain...

ba0001pp74 | Bone development/growth and fracture repair | ECTS2013

Metaphyseal fracture healing in a sheep model of low turnover osteoporosis induced by hypothalamic–pituitary disconnection

Bindl Ronny , Oheim Ralf , Pogoda Pia , Beil Frank Timo , Gruchenberg Katharina , Reitmaier Sandra , Wehner Tim , Calcia Enrico , Radermacher Peter , Claes Lutz , Amling Michael , Ignatius Anita

We recently established a large animal model of osteoporosis in sheep using hypothalamic–pituitary disconnection (HPD). As central regulation is important for bone metabolism, HPD-sheep developed severe osteoporosis because of low bone turnover. In this study we investigated metaphyseal fracture healing in HPD-sheep. To elucidate potential pathomechanisms, we included a treatment group receiving thyroxine (T4) and 17β-estradiol. Because clinically osteopor...

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 ...

ba0004oc10 | (1) | ICCBH2015

Lack of PEDF within the bone matrix is associated with osteoidosis and abnormally high bone mineral content

Fratzl-Zelman Nadja , Schmidt Ingo , Roschger Paul , Roschger Andreas , Reich Adi , Glorieux Francis H. , Wagermaier Wolfgang , Fratzl Peter , Klaushofer Klaus , Marini Joan C. , Rauch Frank

Background and methods: Pigment epithelium-derived factor (PEDF) is a potent antiangiogenic factor, ubiquitously expressed and secreted in human tissues. Hypertrophic cartilage and osteoblasts express PEDF that binds to type I collagen and glycosaminoglycans in the extracellular matrix. Two rare forms of osteogenesis imperfecta (OI) with intact collagen synthesis are associated with PEDF deficiency. Histological observations revealed excessive osteoid formation and prolonged m...

ba0005oc1.5 | Clinical trials and osteoporosis treatment | ECTS2016

Secular trends in prescription incidence of different anti-osteoporotic drugs in the UK population aged 50 years or above from 1990 till 2012

van der Velde Robert , Wyers Caroline , Geusens Piet , van den Bergh Joop , de Vries Frank , Cooper Cyrus , vandeStaa Tjeerd-Pieter , Harvey Nicholas

Objective: To study the trends in prescription of different anti-osteoporotic drugs (AOD) in the UK population aged 50 years or above from 1990 till 2012.Methods: Retrospective observational study using the CPRD data link representative for the total UK population. The incidence of prescription of a specific AOD was calculated by dividing the number of prescriptions by the total person-years (py) of follow-up.Results: AOD prescript...