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Tooth agenesis, selective, 8(STHAG8)

MedGen UID:
934697
•Concept ID:
C4310730
•
Disease or Syndrome
Synonym: STHAG8
 
Gene (location): WNT10B (12q13.12)
 
Monarch Initiative: MONDO:0014901
OMIM®: 617073

Definition

Any tooth agenesis in which the cause of the disease is a mutation in the WNT10B gene. [from MONDO]

Clinical features

From HPO
Microdontia
MedGen UID:
66008
•Concept ID:
C0240340
•
Congenital Abnormality
Decreased size of the teeth, which can be defined as a mesiodistal tooth diameter (width) more than 2 SD below mean. Alternatively, an apparently decreased maximum width of tooth.
See: Feature record | Search on this feature
Sparse eyebrow
MedGen UID:
371332
•Concept ID:
C1832446
•
Finding
Decreased density/number of eyebrow hairs.
See: Feature record | Search on this feature
Selective tooth agenesis
MedGen UID:
370882
•Concept ID:
C1970308
•
Congenital Abnormality
Agenesis specifically affecting one of the classes incisor, premolar, or molar.
See: Feature record | Search on this feature
Dry skin
MedGen UID:
56250
•Concept ID:
C0151908
•
Sign or Symptom
Skin characterized by the lack of natural or normal moisture.
See: Feature record | Search on this feature
Sparse hair
MedGen UID:
1790211
•Concept ID:
C5551005
•
Finding
Reduced density of hairs.
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Developmental defects of enamel and dental caries in the primary dentition: A systematic review and meta-analysis.
Costa FS, Silveira ER, Pinto GS, Nascimento GG, Thomson WM, Demarco FF
J Dent 2017 May;60:1-7. Epub 2017 Mar 24 doi: 10.1016/j.jdent.2017.03.006. PMID: 28347809
Treatment options for congenitally missing lateral incisors.
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Molar root anatomy and management of furcation defects.
Al-Shammari KF, Kazor CE, Wang HL
J Clin Periodontol 2001 Aug;28(8):730-40. doi: 10.1034/j.1600-051x.2001.280803.x. PMID: 11442732

Recent clinical studies

Therapy

Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Prevalence of talon cusp: Systematic literature review, meta-analysis and new scoring system.
Decaup PH, Garot E, Rouas P
Arch Oral Biol 2021 May;125:105112. Epub 2021 Mar 23 doi: 10.1016/j.archoralbio.2021.105112. PMID: 33780721
Surgical removal versus retention for the management of asymptomatic disease-free impacted wisdom teeth.
Ghaeminia H, Perry J, Nienhuijs ME, Toedtling V, Tummers M, Hoppenreijs TJ, Van der Sanden WJ, Mettes TG
Cochrane Database Syst Rev 2016 Aug 31;(8):CD003879. doi: 10.1002/14651858.CD003879.pub4. PMID: 27578151
Treatment options for congenitally missing lateral incisors.
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Evaluation of panoramic radiographs taken from 1,056 Turkish children.
Bekiroglu N, Mete S, Ozbay G, Yalcinkaya S, Kargul B
Niger J Clin Pract 2015 Jan-Feb;18(1):8-12. doi: 10.4103/1119-3077.146965. PMID: 25511336

Prognosis

Multivariate prognostic analysis of direct pulp capping using a bioceramic material in mature permanent teeth with carious pulp exposure: a retrospective cohort study.
Chen J, Liu S, Liu M, Dong Y
Clin Oral Investig 2023 Sep;27(9):5287-5296. Epub 2023 Jul 25 doi: 10.1007/s00784-023-05148-2. PMID: 37490116
Functional Effects of WNT10A Rare Variants Associated with Tooth Agenesis.
Zeng Y, Baugh E, Akyalcin S, Letra A
J Dent Res 2021 Mar;100(3):302-309. Epub 2020 Oct 9 doi: 10.1177/0022034520962728. PMID: 33034246
Clinical and Radiographic Evaluation of a Small-Diameter Dental Implant Used for the Restoration of Patients with Permanent Tooth Agenesis (Hypodontia) in the Maxillary Lateral Incisor and Mandibular Incisor Regions: A 36-Month Follow-Up.
King P, Maiorana C, Luthardt RG, Sondell K, Øland J, Galindo-Moreno P, Nilsson P
Int J Prosthodont 2016 Mar-Apr;29(2):147-53. doi: 10.11607/ijp.4444. PMID: 26929953
Birth-Weight, Pregnancy Term, Pre-Natal and Natal Complications Related to Child's Dental Anomalies.
Prokocimer T, Amir E, Blumer S, Peretz B
J Clin Pediatr Dent 2015 Summer;39(4):371-6. doi: 10.17796/1053-4628-39.4.371. PMID: 26161610
Hemodynamic, ventilator, and ECG changes in pediatric patients undergoing extraction.
Sanadhya YK, Sanadhya S, Jalihal S, Nagarajappa R, Ramesh G, Tak M
J Indian Soc Pedod Prev Dent 2013 Jan-Mar;31(1):10-6. doi: 10.4103/0970-4388.112393. PMID: 23727736

Clinical prediction guides

Does primary trauma lead to developmental defects in permanent teeth?
Gibbison R, Crozier R
Evid Based Dent 2022 Sep;23(3):112-113. Epub 2022 Sep 23 doi: 10.1038/s41432-022-0808-z. PMID: 36151286
Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Prevalence of talon cusp: Systematic literature review, meta-analysis and new scoring system.
Decaup PH, Garot E, Rouas P
Arch Oral Biol 2021 May;125:105112. Epub 2021 Mar 23 doi: 10.1016/j.archoralbio.2021.105112. PMID: 33780721
Functional Effects of WNT10A Rare Variants Associated with Tooth Agenesis.
Zeng Y, Baugh E, Akyalcin S, Letra A
J Dent Res 2021 Mar;100(3):302-309. Epub 2020 Oct 9 doi: 10.1177/0022034520962728. PMID: 33034246
Non-syndromic tooth agenesis patterns and their association with other dental anomalies: A retrospective study.
Souza-Silva BN, Vieira WA, Bernardino ÍM, Batista MJ, Bittencourt MAV, Paranhos LR
Arch Oral Biol 2018 Dec;96:26-32. Epub 2018 Aug 24 doi: 10.1016/j.archoralbio.2018.08.014. PMID: 30172942

Recent systematic reviews

Does primary trauma lead to developmental defects in permanent teeth?
Gibbison R, Crozier R
Evid Based Dent 2022 Sep;23(3):112-113. Epub 2022 Sep 23 doi: 10.1038/s41432-022-0808-z. PMID: 36151286
Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Prevalence of talon cusp: Systematic literature review, meta-analysis and new scoring system.
Decaup PH, Garot E, Rouas P
Arch Oral Biol 2021 May;125:105112. Epub 2021 Mar 23 doi: 10.1016/j.archoralbio.2021.105112. PMID: 33780721
Treatment options for congenitally missing lateral incisors.
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Prevalence of hypodontia and associated factors: a systematic review and meta-analysis.
Khalaf K, Miskelly J, Voge E, Macfarlane TV
J Orthod 2014 Dec;41(4):299-316. doi: 10.1179/1465313314Y.0000000116. PMID: 25404667

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