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Hereditary dentin defects with systemic diseases.

Tongyu Su1, Yulong Zhu1, Xiangpu Wang1

  • 1State Key Laboratory of Military Stomatology, National Clinical Research Center for Oral Diseases, Shaanxi Key Laboratory of Stomatology, Department of Oral Biology & Clinic of Oral Rare and Genetic Diseases, School of Stomatology, The Fourth Military Medical University, Xi'an, China.

Oral Diseases
|April 24, 2023
PubMed
Summary
This summary is machine-generated.

Syndromic dentin defects, often linked to bone disorders, provide key diagnostic clues for dentists. Understanding these conditions enhances knowledge of dentin development and related systemic diseases.

Keywords:
bonedentin dysplasiadentinogenesis imperfectahereditary diseasesystemic disease

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Area of Science:

  • Genetics
  • Dentistry
  • Pathology

Background:

  • Syndromic dentin defects are manifestations of systemic diseases.
  • Understanding these defects improves diagnosis of underlying conditions.

Purpose of the Study:

  • To review recent advancements in syndromic dentin defects.
  • To clarify the relationship between systemic diseases and dentin malformations.
  • To identify involved molecules and mechanisms.

Main Methods:

  • Comprehensive literature search of genetic diseases with dentin malformations.
  • Analysis and comparison of clinical phenotypes and genetic backgrounds.

Main Results:

  • Over 10 systemic diseases, primarily bone disorders, were analyzed.
  • Pathogenic genes often regulate both bone and dentin development.
  • Phenotypes vary, mimicking dentinogenesis imperfecta or presenting unique abnormalities.

Conclusions:

  • Specific dentin defects can be diagnostic phenotypes for dentists.
  • Mechanistic studies offer insights into isolated dentin defects and general dentin development.