amelogenesis imperfecta type 1G

MeSH: C538241ORPHA: 1031

Overview

amelogenesis imperfecta that has material basis in homozygous or compound heterozygous mutation in the FAM20A gene on chromosome 17q24

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with amelogenesis imperfecta type 1G, sourced from HPO and Orphanet clinical annotations.

NephropathyNephrocalcinosisGingival overgrowthAbnormal dental enamel morphologyDelayed eruption of teethAmelogenesis imperfectaYellow-brown discoloration of the teethAbnormality of dental colorIncreased circulating osteocalcin levelAbnormal circulating calcium-phosphate regulating hormone concentrationRenal insufficiencyGingival fibromatosisEnuresisHypocalciuriaElevated circulating alkaline phosphatase concentrationPulp calcificationImpaired renal concentrating abilityImpacted toothHypophosphaturiaHypocitraturiaMacroglossiaPeriodontitisAnterior open-bite malocclusionTooth agenesisDecreased serum creatinineSupernumerary tooth

Classification & Codes

MeSH Code

C538241

Orphanet Code

ORPHA:1031
amelogenesis imperfecta type 1G
MeSHC538241
OrphanetORPHA:1031
Treatments0 drug(s)
Symptoms on record26 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO