Searchable abstracts of presentations at key conferences on calcified tissues

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 ...

ba0004p130 | (1) | ICCBH2015

Chronic recurrent multifocal osteomyelitis: the value of whole-body MRI in a series of 34 children

Jacobs Benjamin , Brown Mathew , Guha Ananya , Alexandrou Dion , Paul O'Donnell , Keen Richard

Objectives: To assess the role of whole-body MRI in the diagnosis and management of chronic recurrent multifocal osteomyelitis (CRMO) is a benign and non-infective auto-inflammatory bone disorder characterised by multiple and recurrent inflammatory bone lesions. No universal diagnostic criteria exist.Methods: Retrospective review of CRMO cases diagnosed at this hospital between 2008 and 2014. Cases were identified from patient records and clinical inform...

ba0004p135 | (1) | ICCBH2015

Chronic recurrent multifocal osteomyelitis in children: a new, MRI-based method of quantifying inflammation in the bone

Alexandrou Dionysios , Jacobs Benjamin , Paul O'Donnell , Hargunani Rikin , Guha Ananya , Roettger Diana , Kubassova Olga

Objectives: The main aim of the study is to describe a new method of quantitative, MRI-based assessment of inflammation in children with chronic recurrent multifocal osteomyelitis (CRMO).General hypothesis: The main hypothesis is that the bone marrow edema, as detected in CRMO with MRI, can be quantified to aid early diagnosis and help identify clinically silent lesions.Methods: We performed a retrospective analysis of static MRI s...

ba0004p144 | (1) | ICCBH2015

Hypomagnesaemia due to lead poisoning in the context of a heterozygous CLDN-16 mutation

Ramaswamy Priya , Kurre Malathi , Muller Dominik , Dargan Paul , Gevers Evelien , Allgrove Jeremy

Background: 3 year old boy born to non-consanguineous parents. He was diagnosed to have autism at 2 years of age. He had a history of pica.Presenting problem: He was admitted with severe carpopedal spasms of hands and feet. Investigations revealed severe hypomagnesaemia at 0.26 (0.7–1.0) mmol/l, hypocalcaemia at 1.59 (2.2–2.6) mmol/l, hypokalaemia, hyponatremia and moderately low vitamin D levels. Parathyroid hormone concentration was low. Urin...