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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

Etiology

MSX1 involved selective tooth agenesis and abnormal labial frenum, pedigree, and retrospective study.
Zhu Y, Zhang Y, Dong J, Ruan W, Yang S, Huang P, Duan X
Oral Dis 2023 Nov;29(8):3168-3172. Epub 2022 Dec 20 doi: 10.1111/odi.14459. PMID: 36478500
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
Consecutive tooth agenesis patterns in non-syndromic oligodontia.
Baba R, Sato A, Arai K
Odontology 2022 Jan;110(1):183-192. Epub 2021 Jul 8 doi: 10.1007/s10266-021-00634-z. PMID: 34236541
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
Rare and Common Variants Conferring Risk of Tooth Agenesis.
Jonsson L, Magnusson TE, Thordarson A, Jonsson T, Geller F, Feenstra B, Melbye M, Nohr EA, Vucic S, Dhamo B, Rivadeneira F, Ongkosuwito EM, Wolvius EB, Leslie EJ, Marazita ML, Howe BJ, Moreno Uribe LM, Alonso I, Santos M, Pinho T, Jonsson R, Audolfsson G, Gudmundsson L, Nawaz MS, Olafsson S, Gustafsson O, Ingason A, Unnsteinsdottir U, Bjornsdottir G, Walters GB, Zervas M, Oddsson A, Gudbjartsson DF, Steinberg S, Stefansson H, Stefansson K
J Dent Res 2018 May;97(5):515-522. Epub 2018 Jan 24 doi: 10.1177/0022034517750109. PMID: 29364747Free PMC Article

Diagnosis

Frequency and Risk Factors of Maxillary Sinusitis of Endodontic Origin Evaluated by a Dynamic Navigation and a New Filter of Cone-Beam Computed Tomography.
Estrela CRA, Bueno MR, Estrela MRA, Estrela LRA, Guedes OA, Azevedo BC, Cintra LTA, Estrela C
J Endod 2022 Oct;48(10):1263-1272. Epub 2022 Aug 7 doi: 10.1016/j.joen.2022.07.011. PMID: 35948173
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
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
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
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

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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