Dental Hard Tissue Study In A Hereditary Dentinogenesis Imperfecta Family

Hereditary dentinogenesis imperfecta is a rare autosomal dominant genetic disorder characterized by abnormal dentin structure, leading to impaired dental function and aesthetics. This study collected a family with hereditary dentinogenesis imperfecta to analyze the phenotype of their dental hard tissues, aiming to gain a deeper understanding of the disease characteristics. We collected exfoliated primary anterior teeth from the proband of the family and from healthy control children for scanning electron microscope analysis and hardness testing. Additionally, we performed Micro-CT analysis on primary molars extracted due to caries from the proband of the family. Scanning electron microscope analysis showed that the dentinal tubule structure of the affected teeth was irregular and significantly reduced in number. Hardness testing results showed no significant change in enamel hardness in the patient group, but the dentin hardness was significantly lower compared to the normal group. Micro-CT analysis demonstrated severe occlusion of the pulp chamber in the patients, presenting an irregular reticular structure. This study found that the dental hard tissues in patients with hereditary dentinogenesis imperfecta exhibit significant alterations. Additionally, the occlusion of the pulp chamber complicates endodontic treatment. By analyzing the dental hard tissues, we gained insights into the anatomical characteristics of this disease, thereby providing substantial support for clinical practice.