Bone Abstracts

Osteoclasts are highly specialized cell types, responsible for the resorption of bone matrix. Coordinated with osteoblasts they contribute to a proper bone turnover. An impaired or reduced function of the osteoclast leads to a pathogenic increase of the bone mass and finally osteopetrosis. Lysosomal hydrolases, as exemplied by CTSK and Acp5 (TRAP), are known to play an important role in the function of osteoclasts, and knockout mouse models of these proteins develop a bone res...

Postmenopausal women with forearm fracture have higher cortical porosity and lower trabecular density perhaps due to excessive age-related bone loss1. Remodelling becomes unbalanced and rapid only after ~45 years of age. We therefore proposed that bone fragility in premenopausal women with a forearm fracture originates during growth. At metaphyses, trabeculae emerging from the periphery of the growth plate form cortex by ‘corticalization’)2. We ...

Severe generalised recessive dystrophic epidermolysis bullosa (RDEB) is a rare disorder resulting from loss of function mutations in the type VII collagen gene (COL7A1). Although RDEB is characterised by severe skin blistering and erosions following minor mechanical trauma, it is a multisystem disorder with pubertal delay and low bone mass as part of the many complications.Children with RDEB have been described as having inadequate gains in bone...

Background: We have recently identified interleukin 1B (IL-1B) as a potential biomarker for predicting breast cancer patients at increased risk for developing bone metastasis. In mouse models, IL-1B and its receptor (IL-1R1) are upregulated in breast cancer cells that metastasise to bone compared with cells that do not. We have now investigated whether blocking IL-1R with the clinically licensed antagonist, anakinra, might be a potential treatment for breast cancer and bone me...

We recruited 60 healthy volunteers ages 5 to 21 to perform scan-rescan precision tests on the XtremeCT II. Participants were positioned in a carbon fiber immobilization cast. iPad-based video content was used to facilitate motion-free compliance. Distal radius and tibia scans were acquired starting 2 mm proximal to the proximal margin of the growth plate or growth plate remnant. Diaphyseal radius and tibia scans were centered at an offset from the same landmark, corresponding ...

Children with cystinosis have numerous risk factors for impaired bone accrual. We used state-of-the-art quantitative imaging of bone microarchitecture (HR-pQCT) to measure trabecular and cortical microstructure and bone strength in children and adolescents (5-20yrs) with cystinosis. We enrolled 20 cystinosis patients and recruited 34 healthy age- and gender- matched controls. Distal radius and tibia HR-pQCT scans (XtremeCT II, Scanco Medical) were acquired 2...

The impact of sex and maturation on trabecular (Tb) and cortical (Ct) microarchitecture in children and young adults has not been well established. The new second-generation high-resolution peripheral quantitative CT (HR-pQCT) scanner (XCT II, Scanco Medical) incorporates three important advances to provide greater spatial resolution, direct measures of Tb thickness and spacing and measures in the Ct midshaft. The aim of this study was to identify sex and maturation effects on...

Objectives: Alagille syndrome (ALGS) is an autosomal dominant disorder attributed to mutations in the Notch signaling pathway. Children with ALGS are at increased risk for fragility fracture, but the etiology of this disposition is unknown. Our objective was to characterize bone mass, geometry, and microarchitecture in children with ALGS.Methods: This was a cross-sectional study of 10 children (9 females) ages 8-18 years, with a clinical diagnosis of ALG...

Objectives: To evaluate musculoskeletal trajectories in children with newly diagnosed Crohn’s disease (CD), and to determine whether children treated with anti-tumour necrosis factor-alpha antibody (anti-TNF, TREATED vs NAÏVE) had persistent deficits at two years.Methods: This was a single-centre prospective, observational inception cohort study. Children with CD underwent assessments within 6.5±9.5 days from diagnosis and annually for two...

Introduction: Vitamin D is known to be important for bone health. Still, the significance of serum 25-hydroxy vitamin D concentrations (s-25OHD) for hip fracture risk of the elderly is uncertain. Discordant findings may in part be explained by difficulties of RCTs or large cohort studies to reach both the frail and the healthy elderly. The objectives of this study were to determine the risk of hip fractures of the elderly related to s-25OHD, including both the frail and the he...