Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature

International Journal of Environmental Research and Public Health

Hanna Winiarska¹  Szczepan Cofta¹  Tamara Pawlaczyk-Kamieńska²  Tomasz Kulczyk³ 

[1] Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Szamarzewskiego 82/84, 60-569 Poznań, Poland;Department of Risk Group Dentistry, Poznan University of Medical Sciences, Bukowska 70, 60-812 Poznań, Poland;Section of Dental Radiology, Department of Biomaterials and Experimental Dentistry, Poznan University of Medical Sciences, Bukowska 70, 60-812 Poznań, Poland;

关键词: primary ciliary dyskinesia;    developmental dental anomalies;    dental morphology;   

DOI  :  10.3390/ijerph17124337

来源: DOAJ

【 摘 要 】

Background: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. Methods: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). Results: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. Conclusions: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.

【 授权许可】

Unknown