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Tooth agenesis, selective, 10(STHAG10)

MedGen UID:
1824050
•Concept ID:
C5774277
•
Disease or Syndrome
Synonym: STHAG10
 
Gene (location): TSPEAR (21q22.3)
 
Monarch Initiative: MONDO:0859339
OMIM®: 620173

Definition

Selective tooth agenesis-10 (STHAG10) is characterized by agenesis of multiple teeth of the permanent dentition. Cone-shaped teeth and taurodontism have also been observed (Du et al., 2018; Bowles et al., 2021). For a general phenotypic description and a discussion of genetic heterogeneity of selective tooth agenesis, see STHAG1 (106600). [from OMIM]

Clinical features

From HPO
Low-set ears
MedGen UID:
65980
•Concept ID:
C0239234
•
Congenital Abnormality
Upper insertion of the ear to the scalp below an imaginary horizontal line drawn between the inner canthi of the eye and extending posteriorly to the ear.
See: Feature record | Search on this feature
Microcephaly
MedGen UID:
1644158
•Concept ID:
C4551563
•
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
See: Feature record | Search on this feature
High palate
MedGen UID:
66814
•Concept ID:
C0240635
•
Congenital Abnormality
Height of the palate more than 2 SD above the mean (objective) or palatal height at the level of the first permanent molar more than twice the height of the teeth (subjective).
See: Feature record | Search on this feature
Narrow forehead
MedGen UID:
326956
•Concept ID:
C1839758
•
Finding
Width of the forehead or distance between the frontotemporales is more than two standard deviations below the mean (objective); or apparently narrow intertemporal region (subjective).
See: Feature record | Search on this feature
Oligodontia
MedGen UID:
904670
•Concept ID:
C4082304
•
Congenital Abnormality
The absence of six or more teeth from the normal series by a failure to develop.
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Prevalence of taurodontism in primary mandibular first molars of ethnic Indian children.
Nagaveni NB, Radhika NB
Gen Dent 2012 Sep-Oct;60(5):e335-40. PMID: 23032243
Root resorption diagnosed with cone beam computed tomography after 6 months of orthodontic treatment with fixed appliance and the relation to risk factors.
Makedonas D, Lund H, Gröndahl K, Hansen K
Angle Orthod 2012 Mar;82(2):196-201. Epub 2011 Aug 9 doi: 10.2319/112810-691.1. PMID: 21827236Free PMC Article

Recent clinical studies

Etiology

Evidence of dental agenesis in late pleistocene Homo.
Lacy SA
Int J Paleopathol 2021 Mar;32:103-110. Epub 2021 Jan 29 doi: 10.1016/j.ijpp.2021.01.001. PMID: 33524842
Surgical removal versus retention for the management of asymptomatic disease-free impacted wisdom teeth.
Ghaeminia H, Nienhuijs ME, Toedtling V, Perry J, Tummers M, Hoppenreijs TJ, Van der Sanden WJ, Mettes TG
Cochrane Database Syst Rev 2020 May 4;5(5):CD003879. doi: 10.1002/14651858.CD003879.pub5. PMID: 32368796Free PMC Article
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
Selective Reduction of Talon Cusps- A Case Series.
Leith R, O'Connell AC
J Clin Pediatr Dent 2018;42(1):1-5. Epub 2017 Sep 22 doi: 10.17796/1053-4628-42.1.1. PMID: 28937898
The short face syndrome.
Opdebeeck H, Bell WH
Am J Orthod 1978 May;73(5):499-511. doi: 10.1016/0002-9416(78)90240-3. PMID: 277066

Diagnosis

Patterns of tooth agenesis in individuals with Down syndrome: A secondary analysis using the Tooth Agenesis Code.
Kalf-Scholte SM, Wijk AV, Mayoral Trias A, Valkenburg C
Spec Care Dentist 2024 Nov-Dec;44(6):1718-1730. Epub 2024 Jul 22 doi: 10.1111/scd.13042. PMID: 39039758
An improved diagnostic method for taurodontism and a comparative study on its effectiveness evaluation.
Da Y, Zhang L, Chai Z, Du H, Hao L, Zhang L, Zhang Z, Shen Y
PLoS One 2024;19(5):e0302810. Epub 2024 May 7 doi: 10.1371/journal.pone.0302810. PMID: 38713685Free PMC Article
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
Evidence of dental agenesis in late pleistocene Homo.
Lacy SA
Int J Paleopathol 2021 Mar;32:103-110. Epub 2021 Jan 29 doi: 10.1016/j.ijpp.2021.01.001. PMID: 33524842
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

Therapy

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, Nienhuijs ME, Toedtling V, Perry J, Tummers M, Hoppenreijs TJ, Van der Sanden WJ, Mettes TG
Cochrane Database Syst Rev 2020 May 4;5(5):CD003879. doi: 10.1002/14651858.CD003879.pub5. PMID: 32368796Free PMC Article
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
The WNT10A gene in ectodermal dysplasias and selective tooth agenesis.
Mues G, Bonds J, Xiang L, Vieira AR, Seymen F, Klein O, D'Souza RN
Am J Med Genet A 2014 Oct;164A(10):2455-60. Epub 2014 Apr 3 doi: 10.1002/ajmg.a.36520. PMID: 24700731Free PMC Article
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

Prognosis

Patterns of tooth agenesis in individuals with Down syndrome: A secondary analysis using the Tooth Agenesis Code.
Kalf-Scholte SM, Wijk AV, Mayoral Trias A, Valkenburg C
Spec Care Dentist 2024 Nov-Dec;44(6):1718-1730. Epub 2024 Jul 22 doi: 10.1111/scd.13042. PMID: 39039758
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
Tooth agenesis-related GLI2 and GLI3 genes may contribute to craniofacial skeletal morphology in humans.
Marañón-Vásquez GA, Dantas B, Kirschneck C, Arid J, Cunha A, Ramos AGC, Omori MA, Rodrigues AS, Teixeira EC, Levy SC, Schroeder A, Matsumoto MAN, Proff P, Antunes LAA, Vieira AR, Antunes LS, Küchler EC
Arch Oral Biol 2019 Jul;103:12-18. Epub 2019 May 11 doi: 10.1016/j.archoralbio.2019.05.008. PMID: 31112935
Defective mitochondrial protease LonP1 can cause classical mitochondrial disease.
Peter B, Waddington CL, Oláhová M, Sommerville EW, Hopton S, Pyle A, Champion M, Ohlson M, Siibak T, Chrzanowska-Lightowlers ZMA, Taylor RW, Falkenberg M, Lightowlers RN
Hum Mol Genet 2018 May 15;27(10):1743-1753. doi: 10.1093/hmg/ddy080. PMID: 29518248Free PMC Article
Clinical procedures for revitalization: current knowledge and considerations.
Galler KM
Int Endod J 2016 Oct;49(10):926-36. Epub 2016 Feb 1 doi: 10.1111/iej.12606. PMID: 26715631

Clinical prediction guides

Patterns of tooth agenesis in individuals with Down syndrome: A secondary analysis using the Tooth Agenesis Code.
Kalf-Scholte SM, Wijk AV, Mayoral Trias A, Valkenburg C
Spec Care Dentist 2024 Nov-Dec;44(6):1718-1730. Epub 2024 Jul 22 doi: 10.1111/scd.13042. PMID: 39039758
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
Tooth agenesis-related GLI2 and GLI3 genes may contribute to craniofacial skeletal morphology in humans.
Marañón-Vásquez GA, Dantas B, Kirschneck C, Arid J, Cunha A, Ramos AGC, Omori MA, Rodrigues AS, Teixeira EC, Levy SC, Schroeder A, Matsumoto MAN, Proff P, Antunes LAA, Vieira AR, Antunes LS, Küchler EC
Arch Oral Biol 2019 Jul;103:12-18. Epub 2019 May 11 doi: 10.1016/j.archoralbio.2019.05.008. PMID: 31112935
Defective mitochondrial protease LonP1 can cause classical mitochondrial disease.
Peter B, Waddington CL, Oláhová M, Sommerville EW, Hopton S, Pyle A, Champion M, Ohlson M, Siibak T, Chrzanowska-Lightowlers ZMA, Taylor RW, Falkenberg M, Lightowlers RN
Hum Mol Genet 2018 May 15;27(10):1743-1753. doi: 10.1093/hmg/ddy080. PMID: 29518248Free PMC Article

Recent systematic reviews

Dental anomalies in individuals with osteogenesis imperfecta: a systematic review and meta-analysis of prevalence and comparative studies.
Prado HV, Soares ECB, Carneiro NCR, Vilar ICO, Abreu LG, Borges-Oliveira AC
J Appl Oral Sci 2023;31:e20230040. Epub 2023 Sep 4 doi: 10.1590/1678-7757-2023-0040. PMID: 37672427Free PMC Article
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, Nienhuijs ME, Toedtling V, Perry J, Tummers M, Hoppenreijs TJ, Van der Sanden WJ, Mettes TG
Cochrane Database Syst Rev 2020 May 4;5(5):CD003879. doi: 10.1002/14651858.CD003879.pub5. PMID: 32368796Free PMC Article

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