[The histo- and ultrastructural characteristics of the bone tissue in osteogenesis imperfecta].

Histo- and ultrastructure of bone tissue in 28 persons (2 section observations and 26 cases of bone biopsy analysis) with congenital fragility of the bones and dysosteogenesis were studied. Age of the victims ranged from 6 months to 38 years (20 men and 8 women). Against the background of bone rarefaction and decrease in quality of basic bone substance the quantity of osteocytes for a unit of osseous beam area increases. Electron-microscopic investigation revealed changes in osteoblast structure (3 types detected) by the degree of development of granular endoplasmic reticulum. In the 2nd and 3d type of osteoblasts the decrease in the quantity of elements of granular endoplasmic reticulum was noted which may be connected with disturbance of biosynthetic processes in cell and fibrillogenesis with maturation of collagenous fibrils and their mineralization. Histo- and ultrastructure of the bone in dysosteogenesis are rather typical and in total with clinico-roentgenological data are informative enough for making diagnosis.