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Accessing the Cytotoxicity and Cell Response to Biomaterials

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Published on: July 8, 2021

Dentinogenesis imperfecta associated with osteogenesis imperfecta.

Mina Biria¹, Fatemeh Mashhadi Abbas, Sedighe Mozaffar

¹Department of Pediatric, School of Dentistry, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Dental Research Journal

|November 20, 2012

Summary

This case study highlights dentinogenesis imperfecta (DI) in a child with osteogenesis imperfecta (OI). Early dental care is crucial due to severe enamel disintegration in primary teeth affected by DI.

Keywords:

Brittle bone disease dental anomalies dentinogenesis imperfecta osteogenesis imperfecta

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Area of Science:

Dentistry
Genetics
Pediatrics

Background:

Osteogenesis imperfecta (OI) is a genetic disorder affecting collagen, leading to bone fragility.
Dentinogenesis imperfecta (DI) is an inherited dentin disorder often associated with OI.
This paper details a case of DI co-occurring with OI in a pediatric patient.

Observation:

A 5-year-old child diagnosed with OI presented with dental anomalies.
Clinical examination revealed yellow/brown discoloration, attrition, and Class III malocclusion in primary teeth.
Hypoplastic enamel was noted on permanent molars, with histological confirmation of abnormal dentin.

Findings:

Primary teeth exhibited more severe DI manifestations than permanent teeth.
Enamel disintegration was a significant finding in the affected primary teeth.
Radiographic and histological analyses confirmed DI in the context of OI.

Implications:

Early and regular dental recalls are essential for managing DI in patients with OI.
Understanding the dental implications of OI is critical for comprehensive patient care.
This case underscores the importance of a multidisciplinary approach to managing complex genetic disorders.