MCID: DNT047
MIFTS: 14

Dentinogenesis Imperfecta Type 2

Categories: Rare diseases, Oral diseases, Bone diseases, Genetic diseases

Aliases & Classifications for Dentinogenesis Imperfecta Type 2

MalaCards integrated aliases for Dentinogenesis Imperfecta Type 2:

Name: Dentinogenesis Imperfecta Type 2 53 59
Dentinogenesis Imperfecta, Shields Type 2 53 59
Capdepont Teeth 53 59
Dgi-2 53 59
Di-2 53 59
Dentinogenesis Imperfecta Without Osteogenesis Imperfecta 73
Opalescent Dentin 73

Characteristics:

Orphanet epidemiological data:

59
dentinogenesis imperfecta type 2
Inheritance: Autosomal dominant; Prevalence: 1-5/10000 (Europe); Age of onset: Childhood;

Classifications:

Orphanet: 59  
Rare odontological diseases


External Ids:

Orphanet 59 ORPHA166260
ICD10 via Orphanet 34 K00.5
UMLS via Orphanet 74 C2973527

Summaries for Dentinogenesis Imperfecta Type 2

NIH Rare Diseases : 53 Dentinogenesis imperfecta type 2 is a rare and severe form of dentinogenesis imperfecta, a condition that affects tooth development. People affected by the condition may have weak and discolored teeth. These problems can affect both primary (baby) teeth and permanent teeth. People with this form of dentinogenesis imperfecta have no normal teeth. Sensorineural hearing loss has also been found in some affected people. Dentinogenesis imperfecta type 2 is caused by changes (mutations) in the DSPP gene and is inherited in an autosomal dominant manner. Treatment is usually focused on protecting primary (baby) and then permanent teeth with preformed pediatric crowns and other interventions. The replacement of teeth might be considered in the future with dentures and/or implants.

MalaCards based summary : Dentinogenesis Imperfecta Type 2, also known as dentinogenesis imperfecta, shields type 2, is related to dentinogenesis imperfecta 1 and dentin dysplasia, type ii. An important gene associated with Dentinogenesis Imperfecta Type 2 is DSPP (Dentin Sialophosphoprotein).

Related Diseases for Dentinogenesis Imperfecta Type 2

Diseases in the Dentinogenesis Imperfecta family:

Dentinogenesis Imperfecta 1 Dentinogenesis Imperfecta Type 2
Dentinogenesis Imperfecta Type 3

Diseases related to Dentinogenesis Imperfecta Type 2 via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 dentinogenesis imperfecta 1 11.3
2 dentin dysplasia, type ii 11.1
3 dentinogenesis imperfecta 10.0

Symptoms & Phenotypes for Dentinogenesis Imperfecta Type 2

Drugs & Therapeutics for Dentinogenesis Imperfecta Type 2

Search Clinical Trials , NIH Clinical Center for Dentinogenesis Imperfecta Type 2

Genetic Tests for Dentinogenesis Imperfecta Type 2

Anatomical Context for Dentinogenesis Imperfecta Type 2

Publications for Dentinogenesis Imperfecta Type 2

Articles related to Dentinogenesis Imperfecta Type 2:

# Title Authors Year
1
Complete overdenture fabrication for a 12-year-old child with dentinogenesis imperfecta type 2. ( 29964251 )
2018

Variations for Dentinogenesis Imperfecta Type 2

Expression for Dentinogenesis Imperfecta Type 2

Search GEO for disease gene expression data for Dentinogenesis Imperfecta Type 2.

Pathways for Dentinogenesis Imperfecta Type 2

GO Terms for Dentinogenesis Imperfecta Type 2

Sources for Dentinogenesis Imperfecta Type 2

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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