Dentinogenesis Imperfecta
Pronunciation: den-tih-noh-JEN-eh-sis im-per-FEK-tah
A hereditary developmental disorder of dentin formation that results in discoloured, translucent, and structurally weakened teeth.
Full Definition
Dentinogenesis imperfecta (DI) is an autosomal dominant condition caused by mutations in the DSPP gene, resulting in abnormal dentin matrix production. The condition presents with opalescent, blue-grey or amber-coloured teeth that are prone to rapid attrition and fracture. In research literature it is classified into three types (Shields Types I, II, and III), and editors must ensure the correct type designation is consistently applied throughout a manuscript. The condition is distinct from dentin dysplasia, which is a related but separate diagnosis. Editors should note that 'dentinogenesis imperfecta' is never hyphenated and should be italicised only when used in the context of a formal taxonomic or genetic classification.
Usage
Usage note: Do not hyphenate. Distinguish from 'amelogenesis imperfecta,' which affects enamel, not dentin. Type designations (I, II, III) use Roman numerals per clinical convention.
In Context
- "Children diagnosed with dentinogenesis imperfecta Type II exhibited significantly higher rates of posterior tooth fracture than controls." — Paediatric dentistry research article
- "The authors should clarify whether the cohort included cases of dentinogenesis imperfecta or dentin dysplasia, as these are distinct diagnoses." — Peer reviewer comment