Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p60 | (1) | ICCBH2019

FGF23-expressing osteocytes are confined to bone packets that completed primary mineralization in patients with chronic kidney disease on dialysis (CKD5D)

Fratzl-Zelman Nadja , Pereira Renata C , Roschger Paul , Salusky Isidro B , Klaushofer Klaus , Wesseling-Perry Katherine

Objectives: FGF23 is expressed in clusters of osteocytes at the trabecular periphery suggesting that FGF23-expressing osteocytes are confined to specific basic multicellular units (BMUs) at the trabecular surface. Higher numbers of FGF23-expressing osteocytes are found in chronic kidney disease (CKD) patients with preserved skeletal mineralization indices. We thus combined immunohistochemistry and quantitative backscattered electron imaging (qBEI) to explore the hypothesis tha...

ba0007p88 | (1) | ICCBH2019

Diagnostic performance of morphometric vertebral fracture analysis (MXA) in children using a 33-point software programme

Alqahtani Fawaz , Crabtree Nicola , Bromiley Paul , Cootes Timothy , Broadley Penny , Lang Isla , Offiah Amaka C

Background: There is significant inter and intraobserver variability in diagnosing vertebral fractures in children. We aimed to evaluate the diagnostic accuracy of morphometric vertebral fracture analysis (MXA) using a 33-point software programme designed for adults, on dual-energy x-ray absorptiometry (DXA) images of children.Methods: Lateral spine DXA images of 420 children aged between 5 and 18 years were retrospectively reviewed. Vertebral fracture a...

ba0007p169 | (1) | ICCBH2019

Rescue diet restores bone matrix mineralization in mice with a non-functioning vitamin D receptor

Misof Barbara , Blouin Stephane , Hartmann Markus , Hofstaetter Jochen , Klaushofer Klaus , Zwerina Jochen , Roschger Paul , Erben Reinhold

Objectives: Mice with a non-functioning vitamin D receptor (VDR mutants) develop severe secondary hyperparathyroidism, which can be rescued by a diet enriched with calcium, phosphate and lactose. In this work, we studied the effects of a low calcium challenge (CD), normal calcium (ND) and a calcium enriched rescue diet (RD) on the bone mineralization density distribution (BMDD) and osteocyte lacunae sections (OLS) in these mice.Methods: BMDD and OLS were...

ba0007p188 | (1) | ICCBH2019

Impact of type 1 diabetes mellitus on skeletal integrity and strength in adolescents aged 12 to 16 years; as assessed by High Resolution peripheral Quantitative Computed Tomography (HRpQCT)

Devaraja Janani , Dimitri Paul , Paggiosi Margaret , Clark Carolyn , Jacques Richard , Bishop Nick

Objectives: To investigate the impact of Type 1 Diabetes Mellitus (T1DM) on cortical and trabecular microarchitecture, and bone strength in adolescents; using High Resolution peripheral Quantitative Computed Tomography (HRpQCT) and microfinite element analysis. To our knowledge, this is the first study in children, assessing the impact of T1DM on skeletal microstructure and strength using HRpQCT.Methods: We recruited 22 patients aged 12–16 years wit...

ba0007p216 | (1) | ICCBH2019

Dual diagnosis of autism and osteogenesis imperfecta: Case examples to illustrate the implications of dual diagnosis for enhanced outcomes for child and family

Jones Rebecca , Seasman Alison , Marr Caroline , Bishop Nicholas , Arundel Paul , Balasubramanian Meena , Team Metabolic Bone

Background: A minority of children with Osteogenesis Imperfecta (OI) seen within the Sheffield National Severe, Complex and Atypical Service (SCAOI) were also identified as showing symptoms consistent with an Autism Spectrum Disorder (ASD) (Balasubramanian et al. 2018). Diagnosis of ASD in conjunction with OI may be delayed due to presenting problems being inappropriately attributed to OI resulting in specialised ASD input not being received by children.<p class="...

ba0007p221 | (1) | ICCBH2019

Speech and hearing impairment and respiratory complications in a large cohort of patients with Achondroplasia

Cocca Alessandra , Irving Melita , Gringras Paul , Turcu Simona , Cheung Moira S

Objectives: Respiratory and Ear, Nose and Throat (ENT) complications are commonly reported in children with achondroplasia but little data is available regarding prevalence and outcome. In an unselected cohort of children with achondroplasia, we studied the prevalence of:•Abnormal cardiorespiratory sleep studies•Speech and hearing impairment</ce:p...

ba0007lb9 | (1) | ICCBH2019

Monitoring skull base abnormalities in children with osteogenesis imperfecta

Wadanamby Shavinthi , Connolly Daniel , Arundel Paul , Bishop Nick J , Offiah Amaka

Objectives: In the context of a lack of national consensus, the aim of this study was to identify the clinical impact of skull base imaging in children with osteogenesis imperfecta (OI).Methods: A retrospective review was conducted of case-notes and radiological images of children with severe, complex and atypical OI at a designated specialist centre, between 01/2012 and 08/2018. Patient demographics, clinical features at time of imaging and radiological...

ba0004is2biog | (1) (1) | ICCBH2015

Bone and osteocyte biology: lessons from human genetic diseases

Lee Brendan

Biographical DetailsDr Brendan Lee is the Robert and Janice McNair Endowed Chair in Molecular and Human Genetics, Professor and Interim Chairman of the Department of Molecular and Human Genetics at Baylor College of Medicine. Dr B Lee co-directs the joint MD Anderson Cancer Center and Baylor College of Medicine Rolanette and Berdon Lawrence Bone Disease Program of Texas, and the Baylor Co...

ba0001oc1.1 | Osteoporosis epidemiology and long term treatment complications | ECTS2013

Disease-specific perception of fracture risk and incident fracture rates among postmenopausal women: findings from the Global Longitudinal Study of Osteoporosis in Women (GLOW)

Gregson Celia , Dennison Elaine , Compston Juliet , Adami Silvano , Adachi Jonathan , Anderson Frederick , Boonen Steven , Chapurlat Roland , Diez-Perez Adolfo , Greenspan Susan , Hooven Frederick , Lacroix Andrea , Nieves Jeri , Netelenbos J Coen , Pfeilschifter Johannes , Rossini Maurizio , Roux Christian , Saag Kenneth , Silverman Stuart , Siris Ethel , Watts Nelson , Wyman Allison , Cooper Cyrus

Patients with improved health understanding have greater autonomy over, and motivation towards, health-related lifestyles. We compared self-perceived fracture risk and 3-year incident fracture rates in postmenopausal women for a range of co-morbid diseases using data from the Global Longitudinal study of Osteoporosis in Women (GLOW).GLOW is an international cohort study involving 723 physician practices across 10 countries in Europe, North America, Austr...

ba0001pp113 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2013

Lower bone turnover in obesity: is there a link to energy metabolism?

Viljakainen Heli , Ivaska Kaisa K , Lipsanen-Nyman Marita , Saukkonen Tero , Andersson Sture , Laitinen Kalevi , Makitie Outi

Background: Observations in rodents suggest that osteocalcin (OC) participates in glucose metabolism. Data from human studies are inconclusive and it remains unclear whether OC is simply a marker of bone turnover (BTM) or if it also mediates interactions between the skeleton and glucose homeostasis. This study determined the responses of BTMs, including OC, to oral glucose tolerance test (OGTT) in obese and normal-weight subjects.Materials and methods: P...