Searchable abstracts of presentations at key conferences on calcified tissues

ba0004oc1 | (1) | ICCBH2015

Abnormal trabecular micro-architecture and bone mechanical properties in adolescent idiopathic scoliosis: a case–control study with high-resolution peripheral quantitative computed tomography and finite element analysis

Cheuk Ka Yee , Chen Huanxiong , Yu Fiona , Lam Tsz Ping , Lee Simon K M , Ng Bobby K W , Cheng Jack C Y

Adolescent idiopathic scoliosis (AIS) is associated with low bone mineral density (BMD) which has been reported to be an important prognostic factor for curve progression. In addition to BMD, high-resolution peripheral quantitative computed tomography (HR-pQCT) allows measurement of volumetric BMD, bone morphology, trabecular micro-architecture, and evaluation with structure model index (SMI) and finite element analysis (FEA) for mechanical property assessment. The aim of this...

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...

ba0004p59 | (1) | ICCBH2015

Vibration treatment can enhance the bioactive response of osteoblasts to vitamin D in adolescent idiopathic scoliosis patients

Zhang Jiajun , Lee WayneY W , Shi Benlong , Tam Elisa M S , Chen Huanxiong , Lee Simon K M , Ng Bobby K W , Cheng Jack C Y , Lam Tsz Ping

Objectives: Adolescent idiopathic scoliosis (AIS) is a complex three-dimensional spinal deformity associated with low bone mass. Our previous clinical trial demonstrated the anabolic bone effect of vibration treatment (VT) at the femoral neck in AIS subjects. The therapeutic effect was more pronounced in those with optimal serum 25(OH) Vit-D level (>40 nmol/l). To investigate possible factor interaction between Vit-D and VT on their anabolic bone effects, this in-vitro...

ba0004p64 | (1) | ICCBH2015

Peripubertal bone quality and density profile of Chinese adolescents in Hong Kong

Cheuk Ka Yee , Cheung Franco , Lee WayneY W , Hung Vivian , Tam Elisa , Lam Tsz Ping , Ng Bobby K W , Lee Simon K M , Cheng Jack C Y

Objectives: This study aimed to evaluate detailed bone profiles during puberty for Chinese adolescents in Hong Kong using high-resolution peripheral quantitative computed tomography (HR-pQCT) and dual-energy x-ray absorptiometry (DXA).Methods: 333 healthy girls and 231 healthy boys (12–16-years-old) were recruited from secondary schools. Areal bone mineral density (aBMD) of bilateral femoral necks were measured by DXA while bone quality parameters w...

ba0004p156 | (1) | ICCBH2015

Children with coeliac disease on gluten free diet have normal bone mass, geometry and muscle mass

Mackinder M , SC Wong , Tsiountsioura M , Shepherd S , Tellemer E , Kyriakou A , Buchanan E , Edwards C , SF Ahmed , P McGrogan , Gersimidis K

Objective: To evaluate musculoskeletal development using pQCT in children with coeliac disease (CD) on gluten free diet (GFD) compared with age and gender matched healthy controlsMethod: 38 children (18 males) with CD on GFD for a duration of 3.6 years (0.6, 12.5) underwent pQCT at 4%, 38 and 66% tibial sites. Bloods were collected in CD children only. Results reported as median (range).Result: Median TTG was 1.8 IU/l (0.1, 114) wi...

ba0005oc6.2 | Development and differentiation (or Aging) | ECTS2016

Bone with uncleavable type I collagen C-propeptide has abnormal development of multiple bone cell populations and increased bone mineral density with age

Barnes Aileen M , Perosky Joseph E , Blouin Stephane , Rajpar M Helen , Khoury Basma , Klaushofer Klaus , Roschger Paul , Fratzl-Zelman Nadja , Kozloff Kenneth M , Marini Joan C

Mutations in the C-propeptide cleavage site of both COL1A1 and COL1A2 cause dominant high bone mass (HBM) osteogenesis imperfecta (OI), characterized by bone hypermineralization. To elucidate the role of C-propeptide processing in bone formation, we generated heterozygous HBM mice in which both residues of the COL1A1 cleavage site were mutated to prevent cleavage by BMP1. HBM mice are smaller than WT in both weight and length and have extremely brittle bones....

ba0005p352 | Osteoporosis: pathophysiology and epidemiology | ECTS2016

Atypical femur fractures (AFF): a case-control study

Imel Erik , Eckert George , Allen Katie , Chandler Julie , Martin Joel , Hui Siu , Johnston C Conrad , DePapp Anne , Santora Art , Choplin Robert , Roth Trenton , Liu Ziyue

Atypical femur fractures (AFF) have been associated with antiresorptive therapy. In a retrospective case-control study, we identified AFF using ASBMR 2013 criteria. Femoral shaft fractures were identified using ICD9 codes. We screened 1479 radiographs. Radiographs were excluded for high-energy trauma, tumor, or periprosthetic fracture. Two radiologists blinded to treatment scored 482 radiographs for AFF features, and jointly adjudicated discrepancies. The required AFF feature,...

ba0006p070 | (1) | ICCBH2017

The abnormally high and heterogeneous bone matrix mineralization after childhood solid organ transplantation is not further increased by bisphosphonate treatment

Fratzl-Zelman Nadja , Valta Helena , Pereira Renata C , Misof Barbara M , Roschger Paul , Jalanko Hannu , Wesseling-Perry Kathrine , Klaushofer Klaus , Makitie Outi

Background: Chronic renal, liver and heart failure in children associate with multiple skeletal complications. Increased fracture incidence often persists after transplantation and might be related to alterations in bone material properties. Moreover, it is not clear whether bisphosphonate therapy (BP) alters bone matrix mineralization in these patients.Methods: In the present study we evaluated bone mineralization density distribution (BMDD) by quantita...

ba0006p138 | (1) | ICCBH2017

Fibrodysplasia ossificans progressiva: baseline characteristics of 101 subjects participating in a global, longitudinal, natural history study

Kaplan Frederick S , Hsiao Edward C , Baujat Genevieve , Brown Matthew A , De Cunto Carmen , Di Rocco Maja , Keen Richard , Al Makkadam Mona , Grogan Donna R , Pignolo Robert J

Objectives: Progressive heterotopic ossification in fibrodysplasia ossificans progressiva (FOP; OMIM #135100) begins in childhood and leads to irreversible restriction of movement, functional impairment, and shortened life-span. Baseline data from an on-going, global, 3-year, natural history study (NHS) describe FOP disease characteristics, and retrospective flare-up history, causes/symptoms, and outcomes.Methods: Data from 101 subjects (recruited from 2...