Searchable abstracts of presentations at key conferences on calcified tissues

ba0003pp25 | Bone biomechanics and quality | ECTS2014

Normal bone matrix mineralization in patients with chronic obstructive pulmonary disease

Misof Barbara , Roschger Paul , Jorgetti Vanda , Klaushofer Klaus , Dempster David , Kulak Carolina

Chronic obstructive pulmonary disease (COPD) has been found associated with low areal bone mineral density and an increase in fracture rate. Previous histomorphometric findings revealed abnormally low cancellous bone volume and thin cortices. In the present work, we studied the same transiliac bone biopsy samples from n=19 COPD patients for cancellous (Cn.) and cortical (Ct.) bone mineralization density distribution (BMDD) based on quantitative backscatter electron im...

ba0003pp125 | Cell biology: osteoblasts and bone formation | ECTS2014

The significance of Bcl-2-associated athanogene-1 (BAG-1)-mediated protein interactions in osteoblast development

Greenhough Joanna , Papadakis Emmanouil , Cutress Ramsey , Townsend Paul , Oreffo Richard , Tare Rahul

BAG-1 interacts with diverse proteins including heat shock proteins (HSC70/ HSP70) and nuclear hormone receptors to modulate cell proliferation, differentiation and apoptosis. We have shown that bone marrow stromal cells (BMSCs) of Bag-1+/− female mice display reduced BMP-2-directed osteogenic differentiation potential. Estradiol binds to estrogen receptors (ERs) and is functionally involved in BMP-directed osteogenic differentiation of osteoprogenito...

ba0003pp174 | Cell biology: osteocytes | ECTS2014

Structural analysis of tooth and jawbone in a type 2 diabetes mouse model

Repp Felix , Kollmannsberger Philip , Roschger Andreas , Roschger Paul , Wagermaier Wolfgang , Fratzl Peter , Weinkamer Richard

In type 2 diabetes mellitus (T2DM) patients, an increased fracture risk is observed, although the bone mineral density is even higher than in non-diabetic patients. This raises the question of the quality of the organic and inorganic matrix in bone1–3. T2DM is also known to forward dysfunctions in the development of soft tissues such as brittle skin due to cross-linking of the collagen or inflammation of the gingiva. For the latter, a possible influence of diab...

ba0003pp220 | Osteoporosis: evaluation and imaging | ECTS2014

The fracture outpatient clinic: what is the additional value of vertebral assessments and which individuals should be more actively recruited?

Muilwijk Carolien , Lips Paul , Lems Willem , Bultink Irene , den Heijer Martin , de Jongh Renate

Fracture outpatient (FO) clinics aim to identify individuals at high fracture risk. Identification of individuals at high fracture risk has been improved since the introduction of vertebral fracture assessment (VFA). Unfortunately, participation rates in FO clinics are often low.The aim of this study is to i) assess the contributory value of VFA in addition to BMD measurements in identifying individuals with high fracture risk; ii) assess characteristics...

ba0003pp252 | Osteoporosis: evaluation and imaging | ECTS2014

The development of a mathematical model to predict the time to osteoporosis using DEXA scanning

Scully Paul , Peters Catherine , Carew Sheila , Sheehy Tina , Costello Aine , Connor Margaret O , Lyons Declan

Background: Dual-energy X-ray absorptiometry (DEXA) is the gold standard used for measuring bone mineral density and such readings are currently used to predict osteoporosis and osteoporotic fractures. However, no similar prediction model has been developed to identify the age that a patient will become osteoporotic based on DEXA scanning.Objective: The aim of this study was to develop a mathematical model to determine the TTO based on two or more DEXA s...

ba0003pp359 | Osteoporosis: treatment | ECTS2014

Ten year alendronate use does not adversely affect bone quality compared to 5 years use: a human iliac crest biopsy study

Hassler Norbert , Gamsjaeger Sonja , Hofstetter Birgit , Brozek Wolfgang , Misof Barbara , Roschger Paul , Klaushofer Klaus , Paschalis Eleftherios

Bisphosphonates (BPs) including alendronate (ALN) are the most widely prescribed therapy for post-menopausal osteoporosis. Despite their overall excellent safety record and efficacy in reducing fractures, concerns have been expressed lately regarding potential detrimental effects due to prolonged bone turnover reduction, although no definite cause–effect relationship has been established to date. The purpose of the present study was to determine bone material quality by R...

ba0003pp407 | Paediatric bone disease | ECTS2014

Glucocorticoid-treated boys with Duchenne muscular dystrophy DMD and osteoporosis have higher bone matrix mineralization before and after i.v. bisphosphonate therapy

Roschger Paul , Misof Barbara M. , Klaushofer Klaus , Rauch Frank , Ward Leanne M

Duchenne muscular dystrophy (DMD) causes progressive muscle weakness and loss of ambulation. While glucocorticoid (GC) therapy improves motor function, many boys sustain fractures due to osteoporosis. Recently, i.v. bisphosphonate (i.v.BP) therapy has shown promise in the treatment of DMD-related osteoporosis. At the same time, bone histomorphometry revealed lowered bone volume and significant reductions in bone formation pre-i.v.BP treatment, and a further drop after 2 years&...

ba0004oc18 | (1) | ICCBH2015

Improvement in bone manifestations and respiratory status in infants and young children with HPP treated with asfotase alfa: an update on the ENB-010-10 trial

Hofmann Christine , Rockman-Greenberg Cheryl , Harmatz Paul , Moseley Scott , Odrljin Tatjana , Liese Johannes

Objectives: Hypophosphatasia (HPP) is a rare metabolic disease caused by loss-of-function mutation(s) in the gene encoding tissue-nonspecific alkaline phosphatase (TNSALP). HPP in infants is characterized by poor skeletal mineralization, respiratory compromise, and a high risk of mortality. We previously reported improved mineralization and respiratory function in 15 patients enrolled in this second study of asfotase alfa, a bone-targeted recombinant human TNSALP, in infants a...

ba0004p10 | (1) | ICCBH2015

Infantile Blount's disease: histopathologic changes in the proximal tibial metaphysis -- comparison between medial and lateral specimens

Hollman Freek , Vroemen Pascal , Rompa Paul , Moh Prosper , van Rhijn Lodewijk , Weltink Tim , Staal Heleen

Only minor literature on histopathologic changes in Blount’s disease is available. This study presents the histologic findings of biopsies harvested from the medial and lateral part of the proximal tibia during the W/M serrated osteotomy in patients with infantile Blount’s disease, performed in Ghana. In this study it is hypothesized that the medial metaphyseal area in these children will present a different histological morphology compared to the lateral metaphyseal...

ba0004p20 | (1) | ICCBH2015

Bone mineral density in patients with autosomal recessive osteopetrosis after hematopoietic cell transplantation

Petryk Anna , Polgreen Lynda E , Dengel Donald R , Defor Todd E , Orchard Paul

Objectives: Autosomal recessive osteopetrosis (OP) is a rare metabolic bone disease characterized by impaired osteoclast function resulting in defective bone resorption and generalized high bone mineral density (BMD). Excessive bone compromises bone marrow space, leading to marrow failure. The infantile malignant form is typically fatal within the first decade of life. The intermediate form presents later during childhood. Currently, the only potential curative therapy for OP ...