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Dentin dysplasia, type II(DTDP2)

MedGen UID:
96026
•Concept ID:
C0399380
•
Congenital Abnormality
Synonyms: Anomalous Dysplasia of Dentin; Coronal Dentin Dysplasia; Coronal Dentin Dysplasias; Dentin Anomalous Dysplasia; Dentin Anomalous Dysplasias; Dentin Dyspalsia, Shields Type 2; Dentin Dysplasia, Coronal; Dentin Dysplasia, Shields Type II; Dentin Dysplasia, Type II; Dentin Dysplasias, Coronal; Dysplasia, Coronal Dentin; Dysplasia, Pulpal; Dysplasias, Coronal Dentin; Dysplasias, Pulpal; Pulpal Dysplasia; Pulpal Dysplasias
SNOMED CT: Dentine dysplasia - Shield's type II (109494000); Anomalous dysplasia of dentine (109494000); Coronal dentine dysplasia (109494000); Pulpal dysplasia (109494000); Dentin dysplasia, type II (109494000)
 
OMIM®: 125420; 125485

Definition

Dentin dysplasia type II (DD-II) is a rare mild form of dentin dysplasia (DD, see this term) characterized by normal tooth roots but abnormal primary dentition. [from ORPHANET]

Professional guidelines

PubMed

A novel DSPP frameshift mutation causing dentin dysplasia type 2 and disease management strategies.
Yuan M, Zheng X, Xue Y, He Z, Song G, Song Y
Oral Dis 2023 Sep;29(6):2394-2400. Epub 2023 Jan 13 doi: 10.1111/odi.14494. PMID: 36597617

Recent clinical studies

Etiology

The Modified Shields Classification and 12 Families with Defined DSPP Mutations.
Simmer JP, Zhang H, Moon SJH, Donnelly LA, Lee YL, Seymen F, Koruyucu M, Chan HC, Lee KY, Wu S, Hsiang CL, Tsai ATP, Slayton RL, Morrow M, Wang SK, Shields ED, Hu JC
Genes (Basel) 2022 May 12;13(5) doi: 10.3390/genes13050858. PMID: 35627243Free PMC Article

Diagnosis

A novel DSPP frameshift mutation causing dentin dysplasia type 2 and disease management strategies.
Yuan M, Zheng X, Xue Y, He Z, Song G, Song Y
Oral Dis 2023 Sep;29(6):2394-2400. Epub 2023 Jan 13 doi: 10.1111/odi.14494. PMID: 36597617
The Modified Shields Classification and 12 Families with Defined DSPP Mutations.
Simmer JP, Zhang H, Moon SJH, Donnelly LA, Lee YL, Seymen F, Koruyucu M, Chan HC, Lee KY, Wu S, Hsiang CL, Tsai ATP, Slayton RL, Morrow M, Wang SK, Shields ED, Hu JC
Genes (Basel) 2022 May 12;13(5) doi: 10.3390/genes13050858. PMID: 35627243Free PMC Article
A rare case diagnosed as dentin dysplasia type II.
Perlea P, Dragomir C, Bodeanu A, Temelcea AN, Iliescu AA
Rom J Morphol Embryol 2018;59(1):359-365. PMID: 29940650
Frameshift mutations in dentin phosphoprotein and dependence of dentin disease phenotype on mutation location.
Nieminen P, Papagiannoulis-Lascarides L, Waltimo-Siren J, Ollila P, Karjalainen S, Arte S, Veerkamp J, Tallon Walton V, Chimenos Küstner E, Siltanen T, Holappa H, Lukinmaa PL, Alaluusua S
J Bone Miner Res 2011 Apr;26(4):873-80. doi: 10.1002/jbmr.276. PMID: 20949630
Dentin dysplasia, type II, or dentin dysplasia, coronal type.
Giansanti JS, Allen JD
Oral Surg Oral Med Oral Pathol 1974 Dec;38(6):911-7. doi: 10.1016/0030-4220(74)90344-2. PMID: 4531620

Prognosis

A novel DSPP frameshift mutation causing dentin dysplasia type 2 and disease management strategies.
Yuan M, Zheng X, Xue Y, He Z, Song G, Song Y
Oral Dis 2023 Sep;29(6):2394-2400. Epub 2023 Jan 13 doi: 10.1111/odi.14494. PMID: 36597617

Clinical prediction guides

A novel DSPP frameshift mutation causing dentin dysplasia type 2 and disease management strategies.
Yuan M, Zheng X, Xue Y, He Z, Song G, Song Y
Oral Dis 2023 Sep;29(6):2394-2400. Epub 2023 Jan 13 doi: 10.1111/odi.14494. PMID: 36597617
The Modified Shields Classification and 12 Families with Defined DSPP Mutations.
Simmer JP, Zhang H, Moon SJH, Donnelly LA, Lee YL, Seymen F, Koruyucu M, Chan HC, Lee KY, Wu S, Hsiang CL, Tsai ATP, Slayton RL, Morrow M, Wang SK, Shields ED, Hu JC
Genes (Basel) 2022 May 12;13(5) doi: 10.3390/genes13050858. PMID: 35627243Free PMC Article
Dentin dysplasia, type II linkage to chromosome 4q.
Dean JA, Hartsfield JK Jr, Wright JT, Hart TC
J Craniofac Genet Dev Biol 1997 Oct-Dec;17(4):172-7. PMID: 9493074

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