amelogenesis imperfecta
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Pathology
- group of developmental conditions affecting the structure & clinical appearance of the enamel of all or nearly all the teeth in a more or less equal manner
- enamel may be hypoplastic, hypomineralised or both, & affected teeth may be discoloured, sensitive or prone to disintegration
- exists in isolation or associated with other abnormalities as part of a syndrome
- enamel crowns are normal in size & shape
Genetics
- autosomal-dominant hypocalcified amelogenesis imperfecta is associated with defects in FAM83H
More general terms
More specific terms
- amelogenesis imperfecta hypoplastic type 1
- amelogenesis imperfecta hypoplastic type 1B
- amelogenesis imperfecta type 1A
- amelogenesis imperfecta type 1C (amelogenesis imperfecta hypoplastic with or without openbite malocclusion)
- amelogenesis imperfecta type 2A1 (amelogenesis imperfecta hypoplastic with or without openbite malocclusion)
- amelogenesis imperfecta type 2A3
- amelogenesis imperfecta type 4; amelogenesis imperfecta hypomaturation-hypoplastic type with taurodontism