Osteogenesis imperfecta (OI) is a heterogeneous rare connective tissue disorder commonly caused by mutations in the collagen type 1 gene. It is a worldwide extensive disorder regardless of age, gender or ethnic group for a children and adults. Typical clinical features are brittle bone, high frequency of fractures and bone deformities. The other observed signs are blue sclera, dentinogenesis im...

Osteogenesis Imperfecta (OI) is a genetic disorder characterized by bones that break easily, often from little or no apparent cause. In this article, we present a patient suffering from OI, who had concomitant active Behçet’s Disease(BD)with repeated oro-genital ulcers, skin postular eruptions and severe recurrent bilateral uveitis. This patient, is, to our knowledge the first reported case in ...

The molecular defect responsible for a sporadic case of extremely severe (type II/III) osteogenesis imperfecta was investigated. The mutation site was localised in the collagen type I pro alpha 2 mRNA molecules produced by the proband's skin fibroblasts by chemical cleavage of mismatch in heteroduplex nucleic acids. Reverse transcription-polymerase chain reaction DNA amplification, followed by ...

Osteogenesis imperfecta (OI) is a genetic bone pathology with prenatal onset, characterized by brittle bones in response to abnormal collagen composition. There is presently no cure for OI. We previously showed that human first trimester fetal blood mesenchymal stem cells (MSCs) transplanted into a murine OI model (oim mice) improved the phenotype. However, the clinical use of fetal MSC is cons...

Osteogenesis Imperfecta (OI) is a heterogeneous genetic disorder, with features that include increased bone fragility, pathological fractures, blue sclera, dentinogenesis imperfecta and conductive or mixed hearing loss. Clinical variability is wide from children with few fractures and normal stature to children with multiple fractures, long bone deformity, scoliosis and extreme short stature. A...

bone is formed through the processes of endochondral and intramembranous ossification. in endochondral ossification primary mesenchymal cells differentiate to chondrocytes and then are progressively substituted by bone, while in intramembranous ossification mesenchymal stem cells (mscs) differentiate directly into osteoblasts to form bone. the steps of osteogenic proliferation, differentiation,...

objectives: the aim of this study was to histologically compare the regenerative properties of two allografts manufactured by two iranian companies. materials and methods: in this study, four 8-mm defects were produced in the calvaria of 12 rabbits. in three defects, three types of allografts namely itb, cenobone and grafton were placed and one defect served as control. samples were prepared an...

osteogenesis imperfecta (oi) is a genetic disorder characterized by bones that break easily, often from little or no apparent cause. in this article, we present a patient suffering from oi, who had concomitant active beh ç et’s disease(bd)with repeated oro-genital ulcers, skin postular eruptions and severe recurrent bilateral uveitis. this patient, is, to our knowledge the first reported case i...