MCID: RGN005
MIFTS: 26

Regional Odontodysplasia

Categories: Oral diseases, Rare diseases

Aliases & Classifications for Regional Odontodysplasia

MalaCards integrated aliases for Regional Odontodysplasia:

Name: Regional Odontodysplasia 58 54
Odontodysplasia 70
Ghost Teeth 58

Characteristics:

Orphanet epidemiological data:

58
regional odontodysplasia
Inheritance: Not applicable; Age of onset: Childhood; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare odontological diseases


External Ids:

ICD10 via Orphanet 33 K00.4
Orphanet 58 ORPHA83450
UMLS 70 C0206554

Summaries for Regional Odontodysplasia

MalaCards based summary : Regional Odontodysplasia, also known as odontodysplasia, is related to tooth agenesis and hair whorl. An important gene associated with Regional Odontodysplasia is DSPP (Dentin Sialophosphoprotein), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers and Cytoskeleton remodeling Neurofilaments. Related phenotypes are gingival overgrowth and carious teeth

Wikipedia : 73 Regional odontodysplasia is an uncommon developmental abnormality of teeth, usually localized to a... more...

Related Diseases for Regional Odontodysplasia

Graphical network of the top 20 diseases related to Regional Odontodysplasia:



Diseases related to Regional Odontodysplasia

Symptoms & Phenotypes for Regional Odontodysplasia

Human phenotypes related to Regional Odontodysplasia:

58 (show all 24)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 gingival overgrowth 58 Occasional (29-5%)
2 carious teeth 58 Frequent (79-30%)
3 delayed eruption of teeth 58 Frequent (79-30%)
4 gingivitis 58 Frequent (79-30%)
5 abnormality of dental enamel 58 Frequent (79-30%)
6 abnormality of dental color 58 Frequent (79-30%)
7 macrodontia 58 Occasional (29-5%)
8 alveolar ridge overgrowth 58 Occasional (29-5%)
9 tooth abscess 58 Frequent (79-30%)
10 yellow-brown discoloration of the teeth 58 Frequent (79-30%)
11 hypoplasia of dental enamel 58 Frequent (79-30%)
12 multiple unerupted teeth 58 Occasional (29-5%)
13 hypoplasia of teeth 58 Occasional (29-5%)
14 abnormality of primary teeth 58 Frequent (79-30%)
15 mandibular pain 58 Occasional (29-5%)
16 hypocalcification of dental enamel 58 Frequent (79-30%)
17 jaw pain 58 Frequent (79-30%)
18 abnormality of dentin 58 Frequent (79-30%)
19 abnormality of the dental pulp 58 Frequent (79-30%)
20 pulp stones 58 Frequent (79-30%)
21 dental enamel pits 58 Frequent (79-30%)
22 unerupted tooth 58 Frequent (79-30%)
23 shell teeth 58 Frequent (79-30%)
24 short dental roots 58 Occasional (29-5%)

GenomeRNAi Phenotypes related to Regional Odontodysplasia according to GeneCards Suite gene sharing:

26 (show all 14)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.5 VIM
2 Increased shRNA abundance (Z-score > 2) GR00366-A-108 9.5 PAX9
3 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.5 KRT19
4 Increased shRNA abundance (Z-score > 2) GR00366-A-16 9.5 PAX9
5 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.5 KRT19
6 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.5 KRT19 VIM
7 Increased shRNA abundance (Z-score > 2) GR00366-A-189 9.5 VIM
8 Increased shRNA abundance (Z-score > 2) GR00366-A-193 9.5 PAX9
9 Increased shRNA abundance (Z-score > 2) GR00366-A-34 9.5 KRT19
10 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.5 KRT19
11 Increased shRNA abundance (Z-score > 2) GR00366-A-56 9.5 PAX9
12 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.5 VIM
13 Increased shRNA abundance (Z-score > 2) GR00366-A-75 9.5 KRT19
14 Increased shRNA abundance (Z-score > 2) GR00366-A-77 9.5 KRT19

Drugs & Therapeutics for Regional Odontodysplasia

Search Clinical Trials , NIH Clinical Center for Regional Odontodysplasia

Genetic Tests for Regional Odontodysplasia

Anatomical Context for Regional Odontodysplasia

Publications for Regional Odontodysplasia

Articles related to Regional Odontodysplasia:

(show top 50) (show all 181)
# Title Authors PMID Year
1
Regional odontodysplasia involving three quadrants of the jaws: a case report. 61 54
19907727 2010
2
Regional odontodysplasia: morphological, ultrastructural, and immunohistochemical features of the affected teeth, connective tissue, and odontogenic remnants. 61 54
18647509 2008
3
Progressive dental development in regional odontodysplasia. 61 54
17613259 2007
4
Regional odontodysplasia. Report of two cases. 54 61
9540089 1998
5
Root agenesis associated with chronic infection and traumatic curettage of the maxillary sinus. 54 61
8058549 1994
6
Treatment Strategies for Patients with Regional Odontodysplasia: A Presentation of Seven New Cases and a Review of the Literature. 61
32895649 2020
7
[Regional odontodysplasia: a report of three cases]. 61
32634890 2020
8
Regional Odontodysplasia Affecting the Maxilla. 61
30900210 2020
9
Familial oligodontia and regional odontodysplasia associated with a PAX9 initiation codon mutation. 61
30809714 2019
10
Deleterious Variants in WNT10A, EDAR, and EDA Causing Isolated and Syndromic Tooth Agenesis: A Structural Perspective from Molecular Dynamics Simulations. 61
31652981 2019
11
Regional odontodysplasia: An analysis of 161 cases from 1953 to 2017. 61
31337932 2019
12
Oral rehabilitation of a case with regional odontodysplasia using a regenerative approach-A case report and a review of literature. 61
30989685 2019
13
Prevalence of dental anomalies in French orthodontic patients: A retrospective study. 61
30249487 2018
14
Oral and Dental Examination Findings in Beech Martens (Martes foina). 61
30213368 2018
15
Management of regional odontodysplasia: a 10-year-follow-up case report and literature review. 61
30775712 2018
16
Regional Odontodysplasia Crossing the Midline. 61
30345960 2018
17
Mandibular Regional Odontodysplasia in an 8-year-old Boy showing Teeth Disorders, Gubernaculum Tracts, and Altered Bone Fractal Pattern. 61
29991866 2018
18
Cervicofacial infection in a 3-year-old child with regional odontodysplasia: A case report and literature review. 61
29491620 2018
19
Clinical, Radiographic, and Histologic Evaluation of Regional Odontodysplasia: a Case Report with 5-year Follow-up. 61
27284563 2016
20
Role of inflammation in developmental odontogenic pathosis. 61
27194886 2016
21
Regional Odontodysplasia with Generalised Enamel Defect. 61
28097027 2016
22
Inflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority. 61
26752881 2015
23
Ghost teeth: Regional odontodysplasia of maxillary first molar associated with eruption disorders in a 10-year-old girl. 61
26538974 2015
24
Regional odontodysplasia: Report of an unusual case involving mandibular arch. 61
26097362 2015
25
Malformations of the tooth root in humans. 61
26578979 2015
26
Generalized odontodysplasia in a 5-year-old patient with Hallermann-Streiff syndrome: clinical aspects, cone beam computed tomography findings, and conservative clinical approach. 61
25047934 2014
27
Clinical and radiographic findings and usefulness of computed tomographic assessment in two children with regional odontodysplasia. 61
25250179 2014
28
Regional odontodysplasia: literature review and report of an unusual case located in the mandible. 61
24717712 2014
29
Oral pathology quiz #82. Case number 1. Regional odontodysplasia. 61
24812725 2014
30
Regional Odontodysplasia: Report of a case. 61
24724145 2013
31
Single tooth odontodysplasia. Case report. 61
24027896 2013
32
[Odontodysplasia. Simultaneous enamel and dental dysplasia]. 61
23836096 2013
33
Generalised versus Regional Odontodysplasia: Diagnosis, Transitional Management, and Long-Term Followup-A Report of 2 Cases. 61
23476815 2013
34
Regional odontodysplasia: orthodontic treatment and transplantation of premolars. 61
23116512 2012
35
Regional odontodysplasia: a case report. 61
23210194 2012
36
Regional odontodysplasia. A literature review and three case reports. 61
22762183 2012
37
Regional odontodysplasia: a case of progressive tooth development. 61
22918107 2012
38
Early diagnosis of regional odontodysplasia in an infant. 61
22446448 2012
39
Regional odontodysplasia: report of a case. 61
22449506 2012
40
Solitary odontodysplasia: a rare entity. 61
22169839 2011
41
Regional odontodysplasia. 61
22016318 2011
42
Regional odontodysplasia: management of an acute case with a scanning electron microscope. 61
21903559 2011
43
Regional Odontodysplasia Crossing Midline: A Rare Case Report. 61
27672258 2011
44
Regional odontodysplasia. 61
21273724 2010
45
Dental management of oculodentodigital dysplasia: a case report. 61
20587963 2010
46
Oral and maxillofacial pathology case of the month. Regional odontodysplasia (ghost teeth). 61
20629368 2010
47
[An obscure anomaly: regional odontodysplasia]. 61
20446549 2010
48
Step-by-step oral rehabilitation of a generalized odontodysplastic patient with implant-supported prostheses: a clinical report. 61
20386215 2010
49
Case report: Unclassified syndrome involving dental enamel, dentine and lack of tooth eruption. 61
19995511 2009
50
Regional odontodysplasia: An unusual case report. 61
21887003 2009

Variations for Regional Odontodysplasia

Expression for Regional Odontodysplasia

Search GEO for disease gene expression data for Regional Odontodysplasia.

Pathways for Regional Odontodysplasia

Pathways related to Regional Odontodysplasia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.08 VIM KRT19
2
Show member pathways
10.66 VIM KRT19

GO Terms for Regional Odontodysplasia

Cellular components related to Regional Odontodysplasia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.62 VIM KRT19

Molecular functions related to Regional Odontodysplasia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 8.62 VIM KRT19

Sources for Regional Odontodysplasia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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