Dentinogenesis imperfecta: Difference between revisions
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'''Dentinogenesis imperfecta''' ('''DI''') is a [[genetic disorder]] of [[tooth development]]. This condition is a type of dentin dysplasia that causes [[teeth]] to be discolored (most often a blue-gray or yellow-brown color) and translucent giving teeth an opalescent sheen.<ref name=":1" /> |
'''Dentinogenesis imperfecta''' ('''DI''') is a [[genetic disorder]] of [[tooth development]]. This condition is a type of dentin dysplasia that causes [[teeth]] to be discolored (most often a blue-gray or yellow-brown color) and translucent giving teeth an opalescent sheen.<ref name=":1" /> Although genetic factors are the main contributor for the disease, any environmental or systemic upset that impedes calcification or metabolisation of calcium can also result in anomalous dentine. |
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Consequently, teeth also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (deciduous) teeth and permanent teeth. This condition is inherited in an [[autosomal dominant]] pattern, as a result of mutations on chromosome 4q21, in the dentine sialophosphoprotein gene (DSPP).<ref>{{Cite journal|last=Beattie ML, Kim JW, Gong SG, Murdoch-Kinch CA, Simmer JP, Hu JCC|first=|date=2006|title=Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21|url=|journal=J Dent Res|volume=85(4)|pages=329 - 333|via=}}</ref> It is one of the most frequently occurring autosomal dominant feature in humans.<ref>{{Cite journal|last=Thotakura SR, Mah T, Srinivasan R, Takagi Y, Veis A, George A.|first=|date=2000|title=The noncollagenous dentin matrix proteins are involved in dentinogenesis imperfecta type II (DGI-II)|url=|journal=J Dent Res 2000|volume=79(3)|pages=835 - 839|via=}}</ref> Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people. |
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==Types== |
==Types== |
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Type I: |
Type I: DI associated with [[Osteogenesis imperfecta|Osteogenesis Imperfecta]] '''(OI)'''. Type of DI with similar dental abnormalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated [[osteogenesis imperfecta]] is of recessive type.<ref>Ten Cate's Oral Histology, Nanci, Elsevier, 2013, page 15</ref> |
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Type II |
Type II: DI not associated with OI. Occurs in people without other inherited disorders (i.e. [[Osteogenesis imperfecta]]). It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities. Also called hereditary opalescent dentin.<ref name=":0" /> |
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Type III: |
Type III: Brandywine isolate. This type is rare with occurrences only in the secluded populations at Maryland, USA.<ref>{{Cite journal|last=Huth KC, Paschos E, Sagner T, Hickel R|first=|date=2002|title=Diagnostic features and pedodontic-orthodontic management in dentinogenesis imperfecta type II: a case report|url=|journal=Int J Paed Dent|volume=1 2|pages=316 - 321|via=}}</ref><ref>{{Cite journal|last=Pettiette M, Wright JT, Trope M|first=|date=1998|title=Dentinogenesis imperfecta: endodontic implications. Case report|url=|journal=Oral Surg Oral Med Oral Path Oral Radiol Endod|volume=86|pages=733 - 737|via=}}</ref> its predominant characteristic is bell-shaped crowns, especially in the permanent dentition. Unlike Types I and II, it involves teeth with shell-like appearance and multiple pulp exposures.<ref name=":0" /> |
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Mutations in the ''DSPP'' [[gene]] have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta. |
Mutations in the ''DSPP'' [[gene]] have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta. |
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Revision as of 11:56, 7 December 2017
| Dentinogenesis imperfecta | |
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| Specialty | Dentistry  |
Dentinogenesis imperfecta (DI) is a genetic disorder of tooth development. This condition is a type of dentin dysplasia that causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent giving teeth an opalescent sheen.[1] Although genetic factors are the main contributor for the disease, any environmental or systemic upset that impedes calcification or metabolisation of calcium can also result in anomalous dentine.
Consequently, teeth also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (deciduous) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, as a result of mutations on chromosome 4q21, in the dentine sialophosphoprotein gene (DSPP).[2] It is one of the most frequently occurring autosomal dominant feature in humans.[3] Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.
Types
Type I: DI associated with Osteogenesis Imperfecta (OI). Type of DI with similar dental abnormalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type.[4]
Type II: DI not associated with OI. Occurs in people without other inherited disorders (i.e. Osteogenesis imperfecta). It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities. Also called hereditary opalescent dentin.[5]
Type III: Brandywine isolate. This type is rare with occurrences only in the secluded populations at Maryland, USA.[6][7] its predominant characteristic is bell-shaped crowns, especially in the permanent dentition. Unlike Types I and II, it involves teeth with shell-like appearance and multiple pulp exposures.[5]
Mutations in the DSPP gene have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta.
Clinical features
Clinical appearance is variable. However, the teeth usually involved and more severely affected are primary teeth in type I; whereas in type II both the dentitions are equally affected.
The teeth may be gray to yellowish brown. They exhibit translucent or opalescent hue. Enamel is usually lost early due to loss of scalloping at the dentoenamel junction (DEJ). However, the teeth are not more susceptible to dental caries than normal ones.
However, certain patients with dentinogenesis imperfecta will suffer from multiple periapical abscesses apparently resulting from pulpal strangulation secondary to pulpal obliteration or from pulp exposure due to extensive coronal wear. They may need apical surgery to save the involved teeth.[5]
Radiographic features
Type I and II show total obliteration of the pulp chamber.
Type III shows thin dentin and extremely enormous pulp chamber.These teeth are usually known as "shell teeth".
Histology
Dentinal tubules are irregular and are bigger in diameter. Areas of uncalcified matrix are seen. Sometimes odontoblasts are seen in dentin.
Treatment
Preventive and restorative care are important as well as esthetics as a consideration. In most cases, full-coverage crowns are needed for esthetic appearance, as well as to prevent further attrition.[1] Another treatment option is bonding, putting lighter enamel on the weakened enamel of the teeth and with lots of treatments of this bonding, the teeth appear whiter to the eye, but the teeth on the inside and under that cover are still the same. Due to the weakened condition of the teeth, many common cosmetic procedures such as braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they were intended to correct.
Dental whitening (bleaching) is contraindicated although it has been reported to lighten the color of DI teeth with some success; however, because the discoloration is caused primarily by the underlying yellow-brown dentin, this alone is unlikely to produce normal appearance in cases of significant discoloration.[5]
See also
References
- ^ a b Illustrated Dental Embryology, Histology, and Anatomy, Bath-Balogh and Fehrenbach, Elsevier, 2011, page 64
- ^ Beattie ML, Kim JW, Gong SG, Murdoch-Kinch CA, Simmer JP, Hu JCC (2006). "Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21". J Dent Res. 85(4): 329–333.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Thotakura SR, Mah T, Srinivasan R, Takagi Y, Veis A, George A. (2000). "The noncollagenous dentin matrix proteins are involved in dentinogenesis imperfecta type II (DGI-II)". J Dent Res 2000. 79(3): 835–839.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Ten Cate's Oral Histology, Nanci, Elsevier, 2013, page 15
- ^ a b c d American Academy of Pediatric Dentistry, Guideline on Dental Management of Heritable Dental Developmental Anomalies, 2013, http://www.aapd.org/media/Policies_Guidelines/G_OHCHeritable.pdf
- ^ Huth KC, Paschos E, Sagner T, Hickel R (2002). "Diagnostic features and pedodontic-orthodontic management in dentinogenesis imperfecta type II: a case report". Int J Paed Dent. 1 2: 316–321.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Pettiette M, Wright JT, Trope M (1998). "Dentinogenesis imperfecta: endodontic implications. Case report". Oral Surg Oral Med Oral Path Oral Radiol Endod. 86: 733–737.
{{cite journal}}: CS1 maint: multiple names: authors list (link)
This article incorporates public domain text from The U.S. National Library of Medicine