Received March 9, 2004 Abstract Osteogenesis imperfecta is a hereditary disorder of connective tissues characterised by low bone mass and bone fragility. Previous studies demonstrated that cyclical pamidronate therapy is effective in increasing bone density and improving clinical outcomes in children with osteogenesis imperfecta. We report our experience in treating two children with cyclical i...

Distraction osteogenesis is a predictable method for restoring missing tissue prior to implant placement. However, pull from the soft tissue can alter the desired direction of the distraction. This article describes and illustrates techniques that are useful for maintaining the desired vector during alveolar distraction osteogenesis. These methods can prevent the need for further intervention a...

We describe two patients aged 16 and 25 years with osteogenesis imperfecta who sustained displaced fractures of the acetabulum following minor trauma. The femoral heads were deformed by impact against the acetabular margin and both cases underwent surgical reconstruction. The quality of the bone and soft tissues made the operations challenging. There were potential complications specific to ost...

PPARgamma plays a central role in the formation of fat. Regulation of PPARgamma activity depends on numerous factors ranging from dietary ligands to nuclear hormone coactivators and corepressors to oxygen-sensing mechanisms. In addition, the interplay of PPARgamma with other nuclear hormone receptors has implications for the balance between adipogenesis and osteogenesis in mesenchymal stem cell...

BACKGROUND Clinical analysis and genetic testing of a family with osteogenesis imperfecta type IV were conducted, aiming to discuss antenatal genetic diagnosis of osteogenesis imperfecta type IV. MATERIAL/METHODS Preliminary genotyping was performed based on clinical characteristics of the family members and then high-throughput sequencing was applied to rapidly and accurately detect the chan...

Delayed bone healing during distraction osteogenesis negatively affects clinical outcome. In addition to autologous bone grafting, several mechanical, chemical, biologic, and external treatment modalities may be employed to promote bone growth during distraction osteogenesis in the pediatric patient. Mechanical approaches include compressive loading of the distraction regenerate, increased freq...

We have analysed six South African families with osteogenesis imperfecta type I using three DNA polymorphisms associated with the pro alpha 2(I) collagen gene. In four of these families linkage of the pro alpha 2(I) gene and the osteogenesis imperfecta phenotype was suggested, whereas in the remaining two families there was a lack of linkage. No distinct correlation could be made between the ph...

Bone marrow stromal cells maintain the adult skeleton by forming osteoblasts throughout life that regenerate bone and repair fractures. We discovered that subsets of these stromal cells, osteoblasts, osteocytes, and hypertrophic chondrocytes secrete a C-type lectin domain protein, Clec11a, which promotes osteogenesis. Clec11a-deficient mice appeared developmentally normal and had normal hematop...

OBJECTIVE To describe vertical distraction osteogenesis of a scapular flap and removable lip support for oral rehabilitation after surgical creation of an ablative defect of the mandible. CASE REPORT A 70-year-old man who was diagnosed with squamous cell carcinoma of the left lower gingiva underwent segmental mandibulectomy for tumor ablation and reconstruction with a scapular bone flap. To a...

Although cell-matrix adhesive interactions are known to regulate stem cell differentiation, the underlying mechanisms, in particular for direct three-dimensional encapsulation within hydrogels, are poorly understood. Here, we demonstrate that in covalently crosslinked hyaluronic acid (HA) hydrogels, the differentiation of human mesenchymal stem cells (hMSCs) is directed by the generation of deg...