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

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...
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Autor*in:	Tamara Pawlaczyk-Kamieńska [verfasserIn] Hanna Winiarska [verfasserIn] Tomasz Kulczyk [verfasserIn] Szczepan Cofta [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2020

Schlagwörter:	primary ciliary dyskinesia developmental dental anomalies dental morphology

Übergeordnetes Werk:	In: International Journal of Environmental Research and Public Health - MDPI AG, 2005, 17(2020), 4337, p 4337
Übergeordnetes Werk:	volume:17 ; year:2020 ; number:4337, p 4337

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc Journal toc

DOI / URN:	10.3390/ijerph17124337

Katalog-ID:	DOAJ002338165

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author	Tamara Pawlaczyk-Kamieńska
spellingShingle	Tamara Pawlaczyk-Kamieńska misc primary ciliary dyskinesia misc developmental dental anomalies misc dental morphology misc Medicine misc R Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature
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topic_title	Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature primary ciliary dyskinesia developmental dental anomalies dental morphology
topic	misc primary ciliary dyskinesia misc developmental dental anomalies misc dental morphology misc Medicine misc R
topic_unstemmed	misc primary ciliary dyskinesia misc developmental dental anomalies misc dental morphology misc Medicine misc R
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title	Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature
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author_browse	Tamara Pawlaczyk-Kamieńska Hanna Winiarska Tomasz Kulczyk Szczepan Cofta
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title_sort	dental anomalies in rare, genetic ciliopathic disorder—a case report and review of literature
title_auth	Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature
abstract	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.
abstractGer	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.
abstract_unstemmed	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.
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title_short	Dental Anomalies in Rare, Genetic Ciliopathic Disorder—A Case Report and Review of Literature
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