Bone Abstracts

Bisphosphonates are widely used drugs in the fight against osteoclast-mediated bone loss, including osteoporosis and Paget’s disease of bone. The first generation of these potent drugs such as clodronate, a non nitrogen-containing bisphosphonate, has been shown to inhibit osteoclast formation and osteoclastic bone resorption both in vitro and in vivo as well as inducing apoptotic cell death. Recent interest has centred on the effects of more potent nitro...

High Bone Mass (HBM) osteogenesis imperfecta (OI) is caused by dominant mutations in the C-propeptide cleavage site of COL1A1 or COL1A2, characterized by bone hypermineralization. To elucidate the role of C-propeptide processing in bone mineralization and development, we generated heterozygous HBM mice with both residues (Ala-Asp) of the COL1A1 cleavage site substituted (Thr-Asn) to prevent processing by BMP1. Two, 6- and 12-month WT and HBM bones were examin...

Objectives: The present study aims at evaluating bone mineral density (BMD) in a population of children with Neurofibromatosis type I (NF1), with particular focus on changes occurring during growth and pubertal development, trying to understand the magnitude and timing of onset of BMD impairment in this multisystemic and progressive disease, the latter poorly defined so far.Methods: Bone metabolic markers (total calcium, phosphorus, bone alkaline phospha...

Background: Chronic renal, liver and heart failure in children associate with multiple skeletal complications. Increased fracture incidence often persists after transplantation and might be related to alterations in bone material properties. Moreover, it is not clear whether bisphosphonate therapy (BP) alters bone matrix mineralization in these patients.Methods: In the present study we evaluated bone mineralization density distribution (BMDD) by quantita...

Background: Previous studies with small number of boys with Duchenne Muscular Dystrophy (DMD) suggest that growth failure occurs in glucocorticoid naïve (GC) boys.Objective: To evaluate height and longitudinal growth in boys with DMD prior to GC.Method: Retrospective evaluation in boys with DMD with height measurements obtained for clinical purposes. Out of the 91 boys currently managed in Scotland, 51 had at least one height ...

Objective: FOP is a rare, severely disabling disease characterized by episodic flare-ups and accumulation of heterotopic ossification (HO) leading to restricted movement, physical disability, and early death. Data from two Phase 2 interventional studies and one natural history study (NHS) were used to evaluate whether palovarotene could reduce HO following an FOP flare-up.Methods: HO volume at the flare-up site was determined by CT at baseline and 12 wee...

Objective: Autism Spectrum Disorder (ASD) is associated with lower bone mass in children. Physical activity and nutrition influence bone pathophysiology, and differences in these lifestyle factors are observed between children with vs. without ASD; however, whether these factors contribute to bone differences is unknown. We examined if: 1) differences existed in bone mineral density (BMD), content (BMC), or bone geometry in boys with vs without ASD and 2) whether physical acti...

Objectives: To assess lumbar spine (LS) BMD Z-scores and the prevalence of vertebral fractures using DXA lateral vertebral (VFA) assessment in children and adolescents with chronic illnesses on GCs.Methods: All children between the ages of 5 and 17 years with chronic non-malignant illnesses who were on GCs (intravenous or oral) for greater than 3 months duration were evaluated. Study participants were children attending the paediatric sub-specia...

Age is one of the most important factors that influence bone condition. At the age of 40–45 years for women (F) and 50 years for men (M) processes of bone formation and resorption are balanced, then bone resorption predominates.We examined 103 M (20–78 years) and 360 F (20–87 years), residing in the Republic of Karelia. Densitometry was performed for all patients to assess age-related changes in bone mineral density (BMD) at the lumbar spi...

Objectives: Children with Perthes disease may present with altered walking patterns even at the healed stage of the disease. The aim of the study was to assess the walking endurance, fatiguability and gait changes of children with healed Perthes disease, and to determine if walking endurance is associated with hip function and quality of life.Methods: Fifty-one children (38 males) aged 8–16 years with >3 years post-diagnosis of Perthes disease c...