Bone Abstracts

Epigenetic mechanisms regulating osteoblast differentiation are still inadequately described. In a genome wide transcriptional profiling of MC3T3-E1 osteoblastic cell line, we identified RCOR2 as a significantly upregulated gene during a differentiation time-course from proliferative to mature osteoblasts. Similar expression profile of RCOR2 was found in mouse calvarial osteoblasts. RCOR2 belongs to CoREST/RCOR family of proteins that regulate action of lysine-specific histone...

We have shown that the bisphosphonate pamidronate (P) given to children <10d post-burn prevents resorptive bone loss and muscle protein breakdown. We have also shown in vitro that Ca modulates the inflammatory response by altering mononuclear cell chemokine production. We hypothesized that P affects muscle protein breakdown by altering cytokine or chemokine concentration directly or indirectly by lowering blood ionized (i) Ca. We retrospectively analysed biomarker...

FDA guidelines recommend the sheep as large animal model for osteoporosis. Studies of osteoporosis induction use dual energy x-ray absorptiometry (DEXA) to evaluate bone status. Experimentally, the T-score is calculated based on reference bone mineral density (BMD) values of animals before treatment (0 M). Whereas clinically the reference BMD represents values of an independent group of patients around 30 years old. The study hypothesize that the use of additional ind...

Goal: The use of intramedullary telescopic constructs for osteosynthesis in surgical correction of bone deformities in children with systemic skeletal disease can be complicated by delayed bony union, and the structural and functional pathology of bone in patients with these disorders do not always make it possible to avoid displacement of bone fragments and effectively correct the deformity.Methods: Analysis of treatment of 11 patients of femoral and ti...

Objectives: While local gene therapy for bone applications has shown some success in preclinical models, systemic delivery of transgenes to the skeleton remains a considerable challenge. Viral vectors such as adeno-associated viruses (AAVs) have great potential as vectors for systemic transgene delivery and may be adapted for emerging gene editing technologies. Furthermore, AAV vectors can have high efficiency, low immunogenicity, and selective tropism towards different tissue...

Objective: The purpose of this study was to verify the influence of age, body mass and stature on the functionality level of children with Osteogenesis Imperfecta (OI). METHODS: Thirty-eight children (8.21±4.26 years, 19 girls and 52.6% OI type III) were evaluated during their hospitalization for Pamidronate intravenous infusion in the Brazilian Midwest reference hospital for OI treatment (University Hospital of Brasília). Body weight and stature were measured and ca...

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...

Objective: To characterize a group of children with Osteogenesis Imperfecta (OI) followed up at the University Hospital of Brasília (HUB), Brazil.Methods: Data were collected with children and adolescents that were hospitalized in the HUB for intravenous pamidronate infusion treatment. This hospital is an OI reference center of the Midwest region in Brazil. The sample consisted of thirty-eight subjects, of which 50% were female. 52% of the children ...

Objectives: This study aimed to correlate handgrip strength and functionality of children with Osteogenesis Imperfecta (OI).Methods: Thirty-eight children and adolescents with different types of OI were single-timed evaluated during their hospitalization for pamidronate intravenous infusion at the University Hospital of Brasília, Brazil. This hospital is the Brazilian Midwest reference for OI treatment through the national health system. These child...

Chondrosarcomas are cartilage-forming, poorly vascularized tumors. With an estimated annual incidence of 1 in 200 000, they represent the second malignant primary bone tumor of adults after osteosarcoma. These tumors are resistant to chemotherapy and radiotherapy, surgical excision remaining the only therapeutic option. However, very few cell lines and animal models are available, and the mechanisms behind their chemoresistance remain largely unknown. Our goal was to establish...