WordNet

not perfect; defective or inadequate; "had only an imperfect understanding of his responsibilities"; "imperfect mortals"; "drainage here is imperfect"

PrepTutorEJDIC

『完全でない』,欠陥のある / (過去時制で動詞が)未完了の / 未完了時制

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/06/04 15:31:11」(JST)

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Dentinogenesis imperfecta (hereditary Opalescent Dentin) is a genetic disorder of tooth development. This condition causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.

Types

Types of dentinogenesis imperfecta with similar dental formalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type. This is type l.

Type II : 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.

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 deciduous teeth in type 1; whereas in type 2 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 DEJ. However, the teeth are not more susceptible to dental caries than normal ones.

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

One 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.

This article incorporates public domain text from The U.S. National Library of Medicine

UpToDate Contents

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1. 歯の発達障害 developmental defects of the teeth
2. 骨形成不全症：臨床的特徴および診断 osteogenesis imperfecta clinical features and diagnosis
3. 骨形成不全症：管理および予後 osteogenesis imperfecta management and prognosis
4. 児童虐待の整形外科的症状の鑑別診断 differential diagnosis of the orthopedic manifestations of child abuse

English Journal

Phenotype and genotype analyses in seven families with dentinogenesis imperfecta or dentin dysplasia.

Li F1, Liu Y1, Liu H1, Yang J1, Zhang F2, Feng H1.
Oral diseases.Oral Dis.2017 Apr;23(3):360-366. doi: 10.1111/odi.12621. Epub 2017 Jan 24.
PMID 27973701

Dentin Sialoprotein is a Novel Substrate of Matrix Metalloproteinase 9 in vitro and in vivo.

Yuan G1,2, Chen L2,3, Feng J2,4, Yang G1,2, Ni Q5, Xu X6, Wan C1,2, Lindsey M7, Donly KJ2, MacDougall M8, Chen Z1, Chen S2.
Scientific reports.Sci Rep.2017 Feb 14;7:42449. doi: 10.1038/srep42449.
PMID 28195206

Deficiency of the DSPP-cleaving enzymes meprin α and meprin β does not result in dentin malformation in mice.

Arnold P1, Koopmann L2, Peters F3, Birkenfeld F4, Goff SV5, Damm T6, Qin C7, Moali C5, Lucius R2, Becker-Pauly C3.
Cell and tissue research.Cell Tissue Res.2017 Feb;367(2):351-358. doi: 10.1007/s00441-016-2498-3. Epub 2016 Sep 15.
PMID 27628095

Japanese Journal

Bilateral cracked permanent first molars in dentinogenesis imperfecta type II : A case report

WANG Ping,HE Wenxi,NI Longxing
Pediatric dental journal : international journal of Japanese Society of Pediatric Dentistry 21(1), 63-66, 2011-03-31
NAID 10028257210

永久歯列象牙質形成不全症に対する実体顕微鏡およびコーンビームCTを用いた歯内治療学的アプローチ

和達礼子,和達重郎,三輪全三,須田英明
日本歯内療法学会雑誌 = The journal of Japan Endodontic Association 32(1), 19-27, 2011-01-31
NAID 10027850931

Prevalence and distribution of selected developmental dental anomalies in an Indian population

Gupta Saurabh K.,Saxena Payal,Jain Sandhya,Jain Deshraj
Journal of Oral Science 53(2), 231-238, 2011
… These patients were examined for the following developmental dental anomalies: shape anomalies (microdontia, talon cusp, dens evaginatus, fusion, taurodontism), number anomalies (hypodontia, oligodontia, anodontia), structural anomalies (amelogenesis imperfecta, dentinogenesis imperfecta) and positional anomalies (ectopic eruption, rotation, impaction). …
NAID 130000834364