Research Evidence
Peer-reviewed studies linked via MeSH term "Dentinogenesis Imperfecta" from the MEDLINE/PubMed database.
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The genetics of non-syndromic dentinogenesis imperfecta: a systematic review.
[object Object], [object Object], [object Object] · Eur Arch Paediatr Dent · 2025
Dental Management of Genetic Dental Disorders: A Critical Review.
[object Object], [object Object], [object Object] et al. · J Dent Res · 2025
Dental Abnormalities in Osteogenesis Imperfecta: A Systematic Review.
[object Object], [object Object], [object Object] et al. · Calcif Tissue Int · 2024
Pretreatments to bonding on enamel and dentin disorders: a systematic review.
[object Object], [object Object] · Evid Based Dent · 2024
Physiology of dentinogenesis and pathophysiology of dentinogenesis imperfecta: how does it affect dentin structure and biomechanics?
[object Object], [object Object], [object Object] et al. · Acta Biomater · 2025
PMID: 41072594Review
The Role of DSPP in Dentine Formation and Hereditary Dentine Defects.
[object Object], [object Object], [object Object] · Chin J Dent Res · 2024
PMID: 38546516Review
A novel DSPP frameshift mutation causing dentin dysplasia type 2 and disease management strategies.
[object Object], [object Object], [object Object] et al. · Oral Dis · 2023
PMID: 36597617Review
Hereditary dentin defects with systemic diseases.
[object Object], [object Object], [object Object] et al. · Oral Dis · 2023
PMID: 37094075Review
A Review of Dentinogenesis Imperfecta and Primary Dentin Disorders in Dogs.
[object Object], [object Object], [object Object] et al. · J Vet Dent · 2022
PMID: 36113440Review
Dentin dysplasia type I-A dental disease with genetic heterogeneity.
[object Object], [object Object], [object Object] et al. · Oral Dis · 2019
Search all PubMed articles for Dentinogenesis imperfecta
Research data from MEDLINE/PubMed
Signs & Symptoms
Based on Human Phenotype Ontology (HPO) disease-phenotype annotations.
Abnormality of the dental root
Very frequent (80-99%)HP:0006486
Pulp obliteration
Very frequent (80-99%)HP:0006350
Abnormal dental pulp morphology
Frequent (30-79%)HP:0006479
Decreased enamel calcification
Frequent (30-79%)HP:0011084
Dentin anomaly
Frequent (30-79%)HP:0010299
Enamel with tendency to chip
Frequent (30-79%)HP:0025124
Generalized dysplasia of tooth enamel
Frequent (30-79%)HP:0006282
Greyish enamel
Frequent (30-79%)HP:0000683
Ligamentous laxity
Frequent (30-79%)HP:0001382
Teeth with type iii dentinogenesis imperfecta
Frequent (30-79%)HP:0000694
Yellow-brown discolored teeth
Frequent (30-79%)HP:0006286
Absence of a tooth
Occasional (5-29%)HP:0001592
Bruising susceptibility
Occasional (5-29%)HP:0000978
Delayed loss of deciduous teeth
Occasional (5-29%)HP:0006335
Finger joint hypermobility
Occasional (5-29%)HP:0006094
Hyperextensibility at elbow
Occasional (5-29%)HP:0010485
Knee joint hypermobility
Occasional (5-29%)HP:0045086
Underdeveloped dental roots
Occasional (5-29%)HP:0006336
Grey sclerae
Very rare (1-4%)HP:0000592
Hearing impairment
Very rare (1-4%)HP:0000365
Increased bleeding time
Very rare (1-4%)HP:0003010
Related Conditions
Dentinogenesis imperfecta - Shield's type I(child)
Hereditary opalescent dentine(child)
Dentinogenesis imperfecta - Shield's type III(child)
Osteogenesis imperfecta with blue sclerae AND dentinogenesis imperfecta(child)
Dentinogenesis imperfecta, short stature, hearing loss, intellectual disability syndrome(child)
Chondrodysplasia, dentinogenesis imperfecta, joint laxity syndrome(child)
Skeletal dysplasia with wormian bone, multiple fractures, dentinogenesis imperfecta syndrome(child)
Shell teeth(child)
Osteogenesis imperfecta, type IV B(child)
Congenital anomaly of tooth(parent)
Hereditary disorder of tooth(parent)
Autosomal dominant hereditary disorder(parent)
Teeth hard tissue diseases(parent)
Developmental hereditary disorder(parent)
Quick Facts
- SNOMED CT
- 196286005
- UMLS CUI
- C0011436
- Fully Specified Name
- Dentinogenesis imperfecta (disorder)
- Specialists
- 0
- Diagnostic Biomarkers
- 0
- HPO Phenotypes
- 21
Medical Disclaimer
This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider regarding any medical condition or treatment plan.
Clinical content is derived from the SNOMED CT clinical ontology and curated medical knowledge graphs.