LA30.6

ICD-11 MMS 2026-01

Amelogenesis imperfecta

Description

Amelogenesis imperfecta presents with a rare abnormal formation of the enamel or external layer of the crown of teeth. Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel: ameloblastin, enamelin, tuftelin and amelogenin. People afflicted with amelogenesis imperfecta have teeth with abnormal colour: yellow, brown or grey; this disorder can afflict any number of teeth of both dentitions. The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes as well as rapid attrition, excessive calculus deposition, and gingival hyperplasia.

Index terms

  • Amelogenesis imperfecta
  • AI - [amelogenesis imperfecta]
  • Hypocalcified amelogenesis imperfecta
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  • hypomineralisation amelogenesis imperfecta
  • Hypomaturation amelogenesis imperfecta
  • Hypomaturation hypoplastic amelogenesis imperfecta with taurodontism
  • Hypoplastic amelogenesis imperfecta
  • congenital enamel hypoplasia
  • hereditary enamel hypoplasia
  • hypoplasia amelogenesis imperfecta
  • Amelogenesis imperfecta - gingival hyperplasia
  • Amelogenesis imperfecta - cone rod dystrophy
  • Jalili syndrome
  • Amelogenesis imperfecta - nephrocalcinosis

Inclusions

  • Hypocalcified amelogenesis imperfecta
  • Hypomaturation amelogenesis imperfecta

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA30.0 – Anodontia
  • LA30.1 – Hypodontia
  • LA30.2 – Oligodontia
  • LA30.3 – Hyperdontia
  • LA30.4 – Abnormalities of size or form of teeth
  • LA30.5 – Anomalies in tooth resorption or loss
  • LA30.7 – Dentine dysplasia
  • LA30.8 – Dentinogenesis imperfecta
  • LA30.9 – Odontogenesis imperfecta
  • LA30.Y – Other specified structural developmental anomalies of teeth and periodontal tissues
  • LA30.Z – Structural developmental anomalies of teeth and periodontal tissues, unspecified

Foundation entity

Amelogenesis imperfecta – http://id.who.int/icd/entity/1923123066

Part of: LA30: Structural developmental anomalies of teeth and periodontal tissues › 20: Developmental anomalies