Bone Abstracts

Objectives: To evaluate the skeletal profile of paediatric patients with cancer at diagnosis.Methods: Children diagnosed with cancer in our Oncology Centre were recruited during a fifteen-month period and underwent metabolic bone profile and dual-energy X-ray absorptiometry (DXA) at the time of diagnosis. Subsequently, they were divided in two subgroups, according to diagnosis; haematological malignancy vs solid tumour. For comparison, a group of 38 sex ...

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Osteoporotic fractures are very common and represent an enormous unmet medical need. Our group has previously reported that addition of rTNF to the fracture site promotes fracture healing in C57/BL6 mice (Glass et al. PNAS 2011). Using a murine fracture model of endochondral healing, we observed that local addition of rTNF only accelerates fracture repair if administered within the first 24 h following injury. The optimal therapeutic dose is 1 ng. TNF is firs...

Odanacatib (ODN), a selective oral inhibitor of cathepsin K, is in development for the treatment of osteoporosis. In the primary efficacy analysis of the Phase 3, Long-Term ODN Fracture Trial (LOFT; NCT00529373), ODN significantly reduced fracture risk compared with placebo in postmenopausal women with osteoporosis. Pre-specified subgroup analyses evaluated the efficacy of ODN in patient subgroups.Women aged ≥65 years, without baseline radiographic...

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Bruck syndrome, a disorder caused by bi-allelic mutations in either PLOD2 or FKBP10, is characterized by flexion contractures and bone fractures and shows strong clinical overlap with the brittle bone disease Osteogenesis Imperfecta. PLOD2 encodes the Lysyl hydroxylase 2 (LH2) enzyme, which is responsible for the hydroxylation of lysine residues in the type-I collagen telopeptides. This hydroxylation directs cross-linking of the collagen fibrils in t...

Parathyroid hormone (PTH) is used to stimulate bone formation in osteoporotic patients, however concerns have been raised about the quality of the matrix produced since lower levels of total matrix mineral have been reported in osteoporotic and fracture patients treated with PTH. High resolution synchrotron-based Fourier Transform Infrared Microscopy (sFTIRM) was used to determine mineral content in age-matched bone during anabolic PTH treatment, using the simplified lamellar ...

Osteosarcoma is the most common primary bone-derived tumor. However, the pathogenic molecular mechanism(s) underpinning osteosarcoma development and metastasis remain elusive. The polycystins PC1 (polycystin-1; encoded by PKD1) and PC2 (polycystin-2) are central players in mechanotransduction, a process that can influence all steps of the invasion/metastasis cascade. Recent studies from our laboratory provided, for the first time, a direct link between mechanosensing polycysti...

Contemporary elemental imaging techniques are greatly contributing into the bone research. Synchrotron radiation induced confocal micro x-ray fluorescence technique (SR-μXRF) was employed for the analysis, being the most powerful and sufficient tool in detection and characterization of trace element distributions in bone tissue. Manganese (Mn), as a potential contributor into the mechanisms of calcium incorporation into bone tissue is in the spotlight of research. Further...

Background: Current extension in the usage of growth hormone therapy (GHT) has increased the prevalence of bone complications. Legg Calvé Perthes disease (LCPD) is characterized by idiopathic avascular necrosis of the proximal femoral epiphysis. More frequently in boys between 4 and 8 years, LCPD is of unknown ethology. An increased incidence has been stated in case of GH deficiency. There is increasing data that children with LCPD may have a more widespread skeletal diso...