Craniofacial features of cleidocranial dysplasia
Cleidocranial dysplasia (CCD) is an autosomal-dominant malformation syndrome affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/aplastic clavicles, open fontanelles, short stature, retention of primary teeth, delayed eruption of perma...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2017-12-01
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| Series: | Journal of Dental Sciences |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1991790217301009 |
| _version_ | 1818846497636089856 |
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| author | Chin-Yun Pan Yu-Chuan Tseng Ting-Hsun Lan Hong-Po Chang |
| author_facet | Chin-Yun Pan Yu-Chuan Tseng Ting-Hsun Lan Hong-Po Chang |
| author_sort | Chin-Yun Pan |
| collection | DOAJ |
| description | Cleidocranial dysplasia (CCD) is an autosomal-dominant malformation syndrome affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/aplastic clavicles, open fontanelles, short stature, retention of primary teeth, delayed eruption of permanent teeth, supernumerary teeth, and multiple impacted teeth. Treatment of CCD requires a multidisciplinary approach that may include dental corrections, orthognathic surgery and cranioplasty along with management of any complications of CCD. Early diagnosis of this condition enables application of the treatment strategy that provides the best quality of life to such patients. Notably, Runx2 gene mutations have been identified in CCD patients. Therefore, further elucidation of the molecular mechanism of supernumerary teeth formation related to Runx2 mutations may improve understanding of dental development in CCD. The insights into CCD pathogenesis may assist in the development of new treatments for CCD. Keywords: cleidocranial dysplasia, mutation, Runx2, supernumerary teeth |
| first_indexed | 2024-12-19T05:46:30Z |
| format | Article |
| id | doaj.art-323cc38da7824b5aa797df4d256cd4f2 |
| institution | Directory Open Access Journal |
| issn | 1991-7902 |
| language | English |
| last_indexed | 2024-12-19T05:46:30Z |
| publishDate | 2017-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Dental Sciences |
| spelling | doaj.art-323cc38da7824b5aa797df4d256cd4f22022-12-21T20:33:48ZengElsevierJournal of Dental Sciences1991-79022017-12-01124313318Craniofacial features of cleidocranial dysplasiaChin-Yun Pan0Yu-Chuan Tseng1Ting-Hsun Lan2Hong-Po Chang3Department of Orthodontics, Dental Clinics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; School of Dentistry, College of Dental Medicine, Kaohsiung Medical University, Kaohsiung, TaiwanDepartment of Orthodontics, Dental Clinics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; School of Dentistry, College of Dental Medicine, Kaohsiung Medical University, Kaohsiung, TaiwanSchool of Dentistry, College of Dental Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Prosthodontics, Dental Clinics, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanSchool of Dentistry, College of Dental Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Dentistry (Orthodontics), Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan; Corresponding author. School of Dentistry, College of Dental Medicine, Kaohsiung Medical University, 100 Shih-Chuan 1st Road, Kaohsiung 80708, Taiwan.Cleidocranial dysplasia (CCD) is an autosomal-dominant malformation syndrome affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/aplastic clavicles, open fontanelles, short stature, retention of primary teeth, delayed eruption of permanent teeth, supernumerary teeth, and multiple impacted teeth. Treatment of CCD requires a multidisciplinary approach that may include dental corrections, orthognathic surgery and cranioplasty along with management of any complications of CCD. Early diagnosis of this condition enables application of the treatment strategy that provides the best quality of life to such patients. Notably, Runx2 gene mutations have been identified in CCD patients. Therefore, further elucidation of the molecular mechanism of supernumerary teeth formation related to Runx2 mutations may improve understanding of dental development in CCD. The insights into CCD pathogenesis may assist in the development of new treatments for CCD. Keywords: cleidocranial dysplasia, mutation, Runx2, supernumerary teethhttp://www.sciencedirect.com/science/article/pii/S1991790217301009 |
| spellingShingle | Chin-Yun Pan Yu-Chuan Tseng Ting-Hsun Lan Hong-Po Chang Craniofacial features of cleidocranial dysplasia Journal of Dental Sciences |
| title | Craniofacial features of cleidocranial dysplasia |
| title_full | Craniofacial features of cleidocranial dysplasia |
| title_fullStr | Craniofacial features of cleidocranial dysplasia |
| title_full_unstemmed | Craniofacial features of cleidocranial dysplasia |
| title_short | Craniofacial features of cleidocranial dysplasia |
| title_sort | craniofacial features of cleidocranial dysplasia |
| url | http://www.sciencedirect.com/science/article/pii/S1991790217301009 |
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