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Published in: BMC Oral Health 1/2015

Open Access 01-12-2015 | Research article

Dentin dysplasia type I—novel findings in deciduous and permanent teeth

Authors: Xin Ye, Kunyang Li, Ling Liu, Fangfang Yu, Fu Xiong, Yun Fan, Xiangmin Xu, Chunran Zuo, Dong Chen

Published in: BMC Oral Health | Issue 1/2015

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Abstract

Background

Dentin dysplasia type I (DD-I) is a rare autosomal dominant hereditary disorder which seriously affects the root development of teeth, causing spontaneous tooth loss (in teenagers). At present, the study of DD-I focuses on familial and phenotypic analyses and reports regarding the ultrastructural study of DD-I are few. The purpose of this study was to clarify and discuss the clinical, histopathological, and ultrastructural features of the dentin defects in DD-I. In addition, the study further explores the root development and provides clues for uncovering virulent genes associated with the disease.

Methods

We recruited 31 members of a four-generation Chinese family, including eleven with dentin defects. Four permanent teeth and four deciduous teeth were obtained from individuals affected by DD-I. At the same time, two caries-free like-numbered permanent teeth and deciduous teeth served as controls, respectively. Analyses of these teeth were carried out using stereomicroscopy, light microscopy, and scanning and transmission electron microscopy, respectively.

Results

Similar to previous reports, extracted teeth showed typical histopathological and ultrastructural features of DD-I and teeth had short roots with obliterated pulp chambers. Furthermore, several novel discoveries were found in teeth affected by DD-I, including; (1) thinner dentin; (2) larger scalloped dentinoenamel junctions; (3) teardrop-shaped lacunae in the enamel; (4) rodless enamel and (5) irregular collagen fibers.

Conclusions

The results exhibited defined features of DD-I in the family and further confirmed that abnormal dentin structure affected both the deciduous and permanent dentitions. In addition, these findings may contribute to a better understanding of the pathogenesis of DD-I as well as aid in the subclassification of this disease.
Literature
1.
go back to reference Toomarian L, Mashhadiabbas F, Mirkarimi M, Mehrdad L. Dentin dysplasia type I: A case report and review of the literature. J Med Case Rep. 2010;4(4):1–6.PubMedPubMedCentralCrossRef Toomarian L, Mashhadiabbas F, Mirkarimi M, Mehrdad L. Dentin dysplasia type I: A case report and review of the literature. J Med Case Rep. 2010;4(4):1–6.PubMedPubMedCentralCrossRef
3.
go back to reference Shields ED, Bixler D, El-Kafrawy AM. A proposed classification for heritable human dentine defects with a description of a new entity. Arch Oral Biol. 1973;18(4):543–IN7.PubMedCrossRef Shields ED, Bixler D, El-Kafrawy AM. A proposed classification for heritable human dentine defects with a description of a new entity. Arch Oral Biol. 1973;18(4):543–IN7.PubMedCrossRef
4.
go back to reference Barron MJ, McDonnell ST, MacKie I, Dixon MJ. Hereditary dentine disorders: Dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis. 2008;3:31.PubMedPubMedCentralCrossRef Barron MJ, McDonnell ST, MacKie I, Dixon MJ. Hereditary dentine disorders: Dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis. 2008;3:31.PubMedPubMedCentralCrossRef
5.
go back to reference de La Dure-Molla M, Fournier BP, Berdal A. Isolated dentinogenesis imperfecta and dentin dysplasia: Revision of the classification. Eur J Hum Genet. 2015;23(4):445–51.CrossRef de La Dure-Molla M, Fournier BP, Berdal A. Isolated dentinogenesis imperfecta and dentin dysplasia: Revision of the classification. Eur J Hum Genet. 2015;23(4):445–51.CrossRef
6.
go back to reference Da Rós Gonçalves L, Oliveira CAGR, Holanda R, et al. Periodontal status of patients with dentin dysplasia type I: Report of three cases within a family. J Periodontol. 2008;79(7):1304–11.PubMedCrossRef Da Rós Gonçalves L, Oliveira CAGR, Holanda R, et al. Periodontal status of patients with dentin dysplasia type I: Report of three cases within a family. J Periodontol. 2008;79(7):1304–11.PubMedCrossRef
7.
go back to reference Ballschmiede G. Dissertation, Berlin, 1922. Malformations of the jaws and teeth. New York: Oxford University Press; 1930. p. 286. Ballschmiede G. Dissertation, Berlin, 1922. Malformations of the jaws and teeth. New York: Oxford University Press; 1930. p. 286.
8.
go back to reference Rushton MA. A case of dentin dysplasia. Guys Hosp Rep. 1939;89:369–73. Rushton MA. A case of dentin dysplasia. Guys Hosp Rep. 1939;89:369–73.
9.
go back to reference Witkop Jr CJ. Hereditary defects of dentin. Dent Clin N Am. 1975;19(1):25–45.PubMed Witkop Jr CJ. Hereditary defects of dentin. Dent Clin N Am. 1975;19(1):25–45.PubMed
10.
go back to reference Eastman JR, Melnick M, Goldblatt LI. Focal odontoblastic dysplasia: Dentin dysplasia type III? Oral Surg Oral Med Oral Pathol. 1977;44(6):909–14.PubMedCrossRef Eastman JR, Melnick M, Goldblatt LI. Focal odontoblastic dysplasia: Dentin dysplasia type III? Oral Surg Oral Med Oral Pathol. 1977;44(6):909–14.PubMedCrossRef
12.
go back to reference Logan J, Becks H, Silverman S, et al. Dentinal dysplasia. Oral Surg Oral Med Oral Pathol. 1962;15(3):317–33.PubMedCrossRef Logan J, Becks H, Silverman S, et al. Dentinal dysplasia. Oral Surg Oral Med Oral Pathol. 1962;15(3):317–33.PubMedCrossRef
13.
go back to reference Sauk JJ, Lyon HW, Trowbridge HO, et al. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol. 1972;33(5):763–71.PubMedCrossRef Sauk JJ, Lyon HW, Trowbridge HO, et al. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol. 1972;33(5):763–71.PubMedCrossRef
14.
go back to reference Kalk WWI, Batenburg RHK, Vissink A. Dentin dysplasia type I: Five cases within one family. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86(2):175–8.PubMedCrossRef Kalk WWI, Batenburg RHK, Vissink A. Dentin dysplasia type I: Five cases within one family. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86(2):175–8.PubMedCrossRef
16.
go back to reference Shankly PE, Mackie IC, Sloan P. Dentinal dysplasia type I: Report of a case. Int J Paediatr Dent. 1999;9(1):37–42.PubMedCrossRef Shankly PE, Mackie IC, Sloan P. Dentinal dysplasia type I: Report of a case. Int J Paediatr Dent. 1999;9(1):37–42.PubMedCrossRef
17.
go back to reference Coke JM, Del Rosso G, Remeikis N, Van Cura JE. Dentinal dysplasia, Type I: Report of a case with endodontic therapy. Oral Surg Oral Med Oral Pathol. 1979;48(3):262–8.PubMedCrossRef Coke JM, Del Rosso G, Remeikis N, Van Cura JE. Dentinal dysplasia, Type I: Report of a case with endodontic therapy. Oral Surg Oral Med Oral Pathol. 1979;48(3):262–8.PubMedCrossRef
18.
go back to reference Ravanshad S, Khayat A. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia Type 1. Aust Endod J. 2006;32(1):40–2.PubMedCrossRef Ravanshad S, Khayat A. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia Type 1. Aust Endod J. 2006;32(1):40–2.PubMedCrossRef
19.
go back to reference Rocha CT, Nelson-Filho P, Silva LAB, Assed S, Queiroz AM. Variation of dentin dysplasia type I: Report of atypical findings in the permanent dentition. Braz Dent J. 2011;22(1):74–8.PubMed Rocha CT, Nelson-Filho P, Silva LAB, Assed S, Queiroz AM. Variation of dentin dysplasia type I: Report of atypical findings in the permanent dentition. Braz Dent J. 2011;22(1):74–8.PubMed
20.
go back to reference Özer L, Karasu H, Aras K, Tokman B, Ersoy E. Dentin dysplasia type I: Report of atypical cases in the permanent and mixed dentitions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004;98(1):85–90.PubMedCrossRef Özer L, Karasu H, Aras K, Tokman B, Ersoy E. Dentin dysplasia type I: Report of atypical cases in the permanent and mixed dentitions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004;98(1):85–90.PubMedCrossRef
21.
go back to reference Carroll MKO, Duncan WK, Perkins TM. Dentin dysplasia: Review of the literature and a proposed subclassification based on radiographic findings. Oral Surg Oral Med Oral Pathol. 1991;72(1):119–25.CrossRef Carroll MKO, Duncan WK, Perkins TM. Dentin dysplasia: Review of the literature and a proposed subclassification based on radiographic findings. Oral Surg Oral Med Oral Pathol. 1991;72(1):119–25.CrossRef
22.
go back to reference Chen D, Liu J, Li K, Wang G, Sun Y, Zhang J, et al. Screening analysis of the pathogenic gene of Dentin dysplasia type I. J Comput Theor Nanosci. 2014;11(6):1437–42.CrossRef Chen D, Liu J, Li K, Wang G, Sun Y, Zhang J, et al. Screening analysis of the pathogenic gene of Dentin dysplasia type I. J Comput Theor Nanosci. 2014;11(6):1437–42.CrossRef
23.
go back to reference Melnick M, Levin LS, Brady J. Dentin dysplasia type I: A scanning electron microscopic analysis of the primary dentition. Oral Surg Oral Med Oral Pathol. 1980;50(4):335–9.PubMedCrossRef Melnick M, Levin LS, Brady J. Dentin dysplasia type I: A scanning electron microscopic analysis of the primary dentition. Oral Surg Oral Med Oral Pathol. 1980;50(4):335–9.PubMedCrossRef
24.
go back to reference De Coster PJ, Cornelissen M, De Paepe A, Martens LC, Vral A. Abnormal dentin structure in two novel gene mutations [COL1A1, Arg134Cys] and [ADAMTS2, Trp795-to-ter] causing rare type I collagen disorders. Arch Oral Biol. 2007;52(2):101–9.PubMedCrossRef De Coster PJ, Cornelissen M, De Paepe A, Martens LC, Vral A. Abnormal dentin structure in two novel gene mutations [COL1A1, Arg134Cys] and [ADAMTS2, Trp795-to-ter] causing rare type I collagen disorders. Arch Oral Biol. 2007;52(2):101–9.PubMedCrossRef
25.
go back to reference Song Y, Wang C, Peng B, Ye X, Zhao G, Fan M, et al. Phenotypes and genotypes in 2 DGI families with different DSPP mutations. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102(3):360–74.PubMedCrossRef Song Y, Wang C, Peng B, Ye X, Zhao G, Fan M, et al. Phenotypes and genotypes in 2 DGI families with different DSPP mutations. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102(3):360–74.PubMedCrossRef
27.
go back to reference Marshall GW, Balooch M, Gallagher RR, et al. Mechanical properties of the dentinoenamel junction: AFM studies of nanohardness, elastic modulus, and fracture. J Biomed Mater Res. 2001;54(1):87–95.PubMedCrossRef Marshall GW, Balooch M, Gallagher RR, et al. Mechanical properties of the dentinoenamel junction: AFM studies of nanohardness, elastic modulus, and fracture. J Biomed Mater Res. 2001;54(1):87–95.PubMedCrossRef
28.
go back to reference Coster PJ. Dentin disorders: Anomalies of dentin formation and structure. Endod Top. 2009;21(1):41–61.CrossRef Coster PJ. Dentin disorders: Anomalies of dentin formation and structure. Endod Top. 2009;21(1):41–61.CrossRef
29.
go back to reference Suzuki S, Sreenath T, Haruyama N, Honeycutt C, Terse A, Cho A, et al. Dentin sialoprotein and dentin phosphoprotein have distinct roles in dentin mineralization. Matrix Biol. 2009;28(4):221–9.PubMedPubMedCentralCrossRef Suzuki S, Sreenath T, Haruyama N, Honeycutt C, Terse A, Cho A, et al. Dentin sialoprotein and dentin phosphoprotein have distinct roles in dentin mineralization. Matrix Biol. 2009;28(4):221–9.PubMedPubMedCentralCrossRef
30.
go back to reference Wesley RK, Wysocki GP, Mintz SM, Jackson J. Dentin dysplasia type I: Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol. 1976;41(4):516–24.PubMedCrossRef Wesley RK, Wysocki GP, Mintz SM, Jackson J. Dentin dysplasia type I: Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol. 1976;41(4):516–24.PubMedCrossRef
Metadata
Title
Dentin dysplasia type I—novel findings in deciduous and permanent teeth
Authors
Xin Ye
Kunyang Li
Ling Liu
Fangfang Yu
Fu Xiong
Yun Fan
Xiangmin Xu
Chunran Zuo
Dong Chen
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Oral Health / Issue 1/2015
Electronic ISSN: 1472-6831
DOI
https://doi.org/10.1186/s12903-015-0149-9

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