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Related to dentinogenesis imperfecta: amelogenesis imperfecta
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References in periodicals archive ?
Distribution of children with dental anomalies according to gender Dental anomaly Male Female Total (% prevalence) Congenitally missing teeth 20 28 48 (0.52%) Supernumerary 10 15 25 (0.27%) Molar-incisor 11 12 23 (0.25%) hypomineralization Turner hypoplasia 8 6 14 (0.1%) Fluorosis 7 4 11 (0.1%) Odontoma 4 6 10 (0.1%) Fusion 5 4 9 (0.09%) Gemination 3 3 6 (0.06%) Amelogenesis imperfecta 3 2 5 (0.05%) Dens invaginatus 1 2 3 (0.03%) Talon cusp 1 1 2 (0.02%) Taurodontism 2 - 2 (0.02%) Macrodontia - 2 2 (0.02%) Dentinogenesis imperfecta 1 1 2 (0.02%) Dilaceration 1 1 2 (0.02%) Ectopic eruption - 1 1 (0.01%) Microdontia - 1 1 (0.01%)
Dentin dysplasia is a hereditary abnormality of dentin with no correlation to systemic disease that is distinct from dentinogenesis imperfecta. Two phenotypes have been described (Table 5).
We also undertook these studies on patients with type IV OI and dentinogenesis imperfecta. In brief, we cultured skin fibroblasts and assessed the synthesis and secretion of type 1 procollagen relative to type 3 procollagen by protein gel electrophoresis.
In dentinogenesis imperfecta, the enamel chips off easily, apparently because of a lack of physical retention at the dentin-enamel junction.
In Dentinogenesis Imperfecta Type I, the dentin mineralization defects are coupled with Osteogenic Imperfecta related bone diseases characterized by a systemic condition involving bone fragility, blue sclera of the eyes, joint laxity, and hearing impairment.
Bilateral occurrence of this condition is frequently observed and it has been reported in association with taurodontism, microdontia, gemination and dentinogenesis imperfecta. In this patient also dens invaginatus was present bilaterally on maxillary lateral incisors associ-ated with microdontia and dilaceration.
Dentinogenesis imperfecta (DI) is an autosomal dominant genetic disorder characterised by abnormal dentine structure affecting the primary and permanent dentitions [Barron et al., 2008].
Dentinogenesis imperfecta is an uncommon defect in the collagen formation that is transmitted as an autosomal dominant trait.
Some people with OI have brittle, discolored teeth, a condition known as dentinogenesis imperfecta.