Scanning electron microscopy of teeth in dominant osteogenesis imperfecta: support for genetic heterogeneity.
Scanning electron microscopic studies were performed on 25 deciduous and permanent teeth from members of 7 kindreds with dominant nonlethal osteogenesis imperfects (OI). Two families had normal teeth on clinical and radiological examination; five families had blue or brown opalescent teeth with specific radiologic findings. Enamel surfaces and prism organization were normal on all teeth. On fractured surfaces, the dentin tubules of normal teeth from patients with OI were evenly distributed and coursed regularly to the dentin-enamel junction. Opalescent teeth had few tubules and those present were short, narrow, and tortuous. Dentin calcification fronts of normal teeth were composed of many nodules with regularly spaced openings on their surfaces. Calcification fronts of opalescent teeth were composed of irregularly spaced, small nodules, which varied greatly in size and the nodules lacked tubule openings on their surfaces. The results of this study support the concept that at least two dominant forms of OI exist--one in which all individuals with IO have normal teeth, and the other in which all with OI have blue or brown opalescent teeth with characteristic changes on SEM.
D003743:Dental Enamel / Q000648:ultrastructure; D003804:Dentin / Q000648:ultrastructure; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008855:Microscopy, Electron, Scanning; D010013:Osteogenesis Imperfecta / Q000145:classification / Q000235:genetics / Q000473:pathology*; D010641:Phenotype; D014070:Tooth / Q000648:ultrastructure*; D014094:Tooth, Deciduous / Q000648:ultrastructure*