Bone Abstracts

Objective: Evaluate the clinical characteristics of all Latin American pediatric patients with Gaucher disease type 1 (GD1) enrolled in the ICGG Gaucher Registry at baseline and investigate long-term outcomes and clinical benefit of prolonged imiglucerase/alglucerase therapy in patients with manifestations of GD1 at baseline.Methods: All Latin American patients with GD1 in the ICGG Gaucher Registry (NCT00358943) who were <18 years of age at the start...

Bone mineral density (BMD) as measured by quantitative computed tomography (QCT) is biased by the variable marrow composition since marrow fat reduces the apparent BMD. We developed a marrow densitometry (MD) method that identifies subvolumes in the vertebrae that consist of bone marrow only (bone voids) and determines their average mineral equivalent density values. The method was developed for use with in vivo high resolution QCT (HR-QCT). HR-QCT still has limited s...

Purpose: To test whether bisphosphonates for osteoporosis reduce the risk of breast cancer.Background: Observational studies and meta-analyses find that women taking bisphosphonates have about a 1/3rd reduction in risk of breast cancer as soon as 1 year of treatment. This is plausible because bisphosphonates have in vitro anti-tumor activity and high doses of zoledronate reduce recurrence in women with breast cancer. However, observational studi...

High fat diet (HFD), obesity, and physical inactivity characterize the modern lifestyle. This can lead to coronary heart diseases and type 2 diabetes mellitus. Recent studies have shown that these conditions frequently are associated with poor bone quality. However, the molecular mechanisms are poorly understood. To analyze the effect of HFD and exercise (EX) on bone homeostasis, we fed 6-week-old C57BL/6 mice a high fat (60% fat) or standard chow diet for 10 weeks (10–20...

Morbidity and mortality are associated with osteoporosis and sarcopenia. There are few data describing the associations between functional measures of muscle and bone. Therefore, the aim of this study was to examine the associations of muscle strength and tibial bone outcomes in ageing men.Men (n=301) aged 40–85 years were recruited in UK (201-White, 43-Black, 57-South-Asian). pQCT was performed at the 38 and 66% tibia with the outcome meas...

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High fat diet (HFD), obesity, and physical inactivity characterize the modern lifestyle. This can lead to coronary heart diseases and type 2 diabetes mellitus. Recent studies have shown that these conditions frequently are associated with poor bone quality. However, the molecular mechanisms are poorly understood. To analyze the effect of HFD and exercise (EX) on bone homeostasis, we fed 6 week-old C57BL/6 mice a high fat (60% fat) or standard chow diet for 10 weeks (10-20 mice...

An increasing number of biomaterials are in development, seeking to mimic the natural cascade of events during fracture repair. However, these biomaterials need to be rigorously tested prior to clinical application. In vitro testing lacks the physiological environment, while in vivo studies do not always predict the patient response. Here we hypothesize that the chorioallantoic membrane (CAM) assay can be used to culture human living bone and we aim to examin...

Background: Peroxisomal Biogenesis Disorders (PBD) is a group of rare metabolic diseases in which peroxisomal function is disrupted. PBD encompasses Zellweger Syndrome Spectrum (ZSS) disorders, which range in severity from classical ZS with severe neurological impairment and markedly reduced life expectancy to Refsum Disease presenting later in childhood. Recent fragility fractures in our ZSS patients in very early childhood prompted case series review.P...

Background: There is still limited information on changes in growth especially segmental growth and bone mass of glucocorticoid(GC) treated boys with Duchenne Muscular Dystrophy (DMD).Objectives: To evaluate changes in growth and bone mass in GC treated boys with DMD.Methods: Retrospective study of 15 boys with DMD treated with GC, median age 7.6 years (4.1, 15.5) who had repeated DXA scan for clinical monitoring of bone health, me...