Bone Abstracts

Objective: Turner syndrome (TS) is a chromosomal aberration characterized by total or partial loss of one of the two X-chromosomes, and affects about 1 in every 2500 girls. TS patients can develop the bone disease with decreased bone density and selective reduction in cortical bone thickness, which probably contributes to the increased fracture risk. However, the mechanisms underlying the bone disease remain poorly understood. Thus, the aim of this study was to investigate the...

Objectives: To evaluate bone health and its possible correlations to body composition parameters in young patients with galactose metabolic disorders, aiming to suggest appropriate lifestyle interventions.Methods: We studied 22 patients, aged 5–16 years with galactose metabolic disorders, detected by neonatal screening. Fourteen suffered from classic galactosemia and eight from other galactose metabolic disorders (i.e. epimerase or galactokinase def...

Objectives: The ability to discriminate bone fractures that result from non-accidental injuries and those that result from underlying bone fragility is limited by the lack of clinical instrumentation to directly measure bone mechanical properties. The BioDent Hfc (Active Life Scientific) is an experimental device that has been validated for measuring cortical bone fracture resistance in adults. We are currently developing protocols to test the feasibility of its use in infants...

Objectives: To determine the prevalence and intensity of D-deficiency in children and adolescents in the metropolitan area with subtotal deficiency of ultraviolet B (55° N).Methods: From May 2008 to May 2010 in a random sample of 163 Muscovites 0–18 years old (9.9±0.4; girls/boys, 81/82) serum 25-hydroxyvitamin D content was determined by chemiluminescent analysis (DiaSorin, Inc., USA; n=56 and Roche Diagnostics; n=107). ...

There is discussed an interrelation of BMD, progressing of erosive and destructive changes in hands and feet with development of vertebral deformations in rheumatoid arthritis (RA).The aim: To receive data on bone mineral density (BMD), erosive and destructive changes in hands and feet and vertebral deformations in patients with RA.Materials/methods: In this research, it was included 106 women, with RA, age 23–69 years. In all...

The mature human skeleton is a metabolically active organ that is continuously resorbed and rebuilt by osteoclasts and osteoblasts. Dietary supplementation of selected long chain polyunsaturated fatty acids (LCPUFAs) has shown effects on bone turnover. Most research on LCPUFAs has been done using commercially available cell lines and further clarification of the cellular effects on models relevant to humans is required. After ethical approval, peripheral blood was collected fr...

Loss of genomic maintenance contributes to aging, as exemplified by mutations in Ercc DNA repair proteins that lead to a plethora of progeroid syndromes of which some display accelerated bone loss. It is generally accepted that dietary restriction (DR) increases life span and improves organ function. We therefore assessed the impact of DR on life span and bone mass in WT and bona fide prematurely aging hypomorphic Ercc-deficient mice (Ercc1−/Δ).<...

The oim/oim mouse resembles type III OI. Our study mimics clinical questions: is RANK-Fc as efficacious as ALN in reducing fracture number? Is RANK-Fc safe to administer to children? Is there any advantage to receiving RANK-Fc treatment post adolescence, following no treatment or ALN treatment from infancy through adolescence?All animal work was done under an IACUC-approval. Mice – n=200, WT or oim/oim. Treatment spanned a...

Cystic fibrosis(CF) results in low volumetric bone mineral density(vBMD), poor muscle strength and increased fracture risk in young patients. The aim of this study was to compare bone and muscle variables measured by peripheral and high-resolution QCT (pQCT and HR-pQCT) and jumping mechanography (JM) In CF children and healthy controls. We hypothesised that CF children have lower muscle force and power (Fmax and Pmax) than controls which may contribute to CF-related bone disea...

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 ...