Bone Abstracts

Objectives: The objective of this study is to characterize patterns of bone morphology in children with osteogenesis imperfecta (OI), using measurements of the second metacarpal. A secondary objective is to look for impact of bisphosphonate treatment on bone morphology in this population.Methods: This is an IRB-approved retrospective review of 82 de-identified bone age films (AP hand/wrist) for 42 children with OI (17M, 25F). Measurements (Sectra IDS 7 P...

Objective: Investigate bone mineral density in children with cerebral palsy (CP) with special focus on severe forms of CP.Methods: Investigation encompassed 23 children, both genders, between 6 and 17 years of age, with diagnosed cerebral palsy, who were hospitalized between January 1 and December 31, 2014. Bone mineral density (BMD) was established using Dual-energy x-ray Absorptiometry – DXA method, at L1–L4 lumbar vertebrae and at the femur ...

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Extreme high bone mass (HBM) may be monogenic (e.g. due to mutations in SOST or LRP5) or polygenic, due to variants in the same genes determining bone mineral density (BMD) as found in the general population. We aimed to determine the genetic cause underlying HBM in an extreme HBM population.258 unexplained HBM cases (defined as L1 Z-score ≥+3.2 plus total hip Z-score ≥+1.2, or total hip Z-score ≥+3.2 and L1 <...

Fibrous dysplasia (FD) is a benign skeletal disease caused by activating mutations of Gsα. These mutations lead to formation of abnormal and mechanically unsound bone and fibrotic tissue. Clinical sequelae include deformity, fracture, and pain. Studies in bisphosphonates have shown improvement in bone pain and inconsistent effects on FD mineralization; however interpretation has been limited by a lack of controlled trials.Objecti...

Background and methods: Pigment epithelium-derived factor (PEDF) is a potent antiangiogenic factor, ubiquitously expressed and secreted in human tissues. Hypertrophic cartilage and osteoblasts express PEDF that binds to type I collagen and glycosaminoglycans in the extracellular matrix. Two rare forms of osteogenesis imperfecta (OI) with intact collagen synthesis are associated with PEDF deficiency. Histological observations revealed excessive osteoid formation and prolonged m...

Background: The relation between sex hormone-binding globulin (SHBG) and vertebral fracture (VFx) is unclear.Aim: To examine whether SHBG is associated with bone mineral density at lumbar spine (LS-BMD), trabecular bone score (TBS) and prevalent VFx.Methods: Data of 6224 men and women participants in the third visit of the first cohort (I-3) and the first visit of the second cohort (II-1) of a prospective population based cohort, w...

Background: Fracture risk is increased in Type 2 diabetes (T2D) individuals. Bone mineral density (BMD) is inversely associated with fracture risk but paradoxically high in T2D individuals. Trabecular bone score (TBS) reflects bone microarchitecture and predicts fracture risk.Objective: We aimed to compare mean lumbar spine TBS (LS-TBS) and lumbar spine BMD (LS-BMD) values, across individuals with and without i) vertebral fracture (VFx), ii) T2D; and iii...

Background and aim: Burosumab is a monoclonal antibody against anti-FGF23, which has been recently approved for treatment of X-linked hypophosphatemia (XLH). Beyond clinical trials, little is known about its efficacy/safety in clinical practice which is the aim of study.Patients/Methods: 39 children with XLH were switched from conventional therapy to burosumab (starting dose 0.4 mg/kg), because of following indications: non-responder to conventional ther...

Ablation of osteosarcoma cells by sublethal hypothermia before radiation may increase sarcoma tissue sensitivity by inducing growth arrest. Normal cells that are not lethally damaged by hypothermia and radiation can undergo DNA repair thus promoting cell survival. Nevertheless, understanding of the response of normal bone forming osteoblast cells towards hypothermia is necessary before administering on osteosarcoma cells. In this study we evaluated the response of short-term m...