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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.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
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).
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).
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.

Professional guidelines

PubMed

Nagaveni NB, Radhika NB
Gen Dent 2012 Sep-Oct;60(5):e335-40. PMID: 23032243
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

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
Janson G, Maria FR, Bombonatti R
Prog Orthod 2014;15(1):51. Epub 2014 Aug 12 doi: 10.1186/s40510-014-0051-z. PMID: 25139394Free PMC Article
Colletti G, Frigerio A, Giovanditto F, Biglioli F, Chiapasco M, Grimmer JF
J Oral Maxillofac Surg 2014 Jul;72(7):1326.e1-18. Epub 2014 Feb 15 doi: 10.1016/j.joms.2014.02.012. PMID: 24768420
Taniguchi JB, Elui VM, Osório FL, Hallak JE, Crippa JA, Machado-de-Sousa JP, Kebbe LM, Lourenço CM, Scarel-Caminaga RM, Marques W Jr
Arq Neuropsiquiatr 2013 Jun;71(6):392-6. doi: 10.1590/0004-282X20130045. PMID: 23828533
Opdebeeck H, Bell WH
Am J Orthod 1978 May;73(5):499-511. doi: 10.1016/0002-9416(78)90240-3. PMID: 277066

Diagnosis

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
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
Lacy SA
Int J Paleopathol 2021 Mar;32:103-110. Epub 2021 Jan 29 doi: 10.1016/j.ijpp.2021.01.001. PMID: 33524842
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
Filo K, Schneider T, Kruse AL, Locher M, Grätz KW, Lübbers HT
Swiss Dent J 2015;125(3):278-92. doi: 10.61872/sdj-2015-03-149. PMID: 26168686

Therapy

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
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
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
Sindet-Pedersen S, Enemark H
J Oral Maxillofac Surg 1990 Jun;48(6):554-8; discussion 559-60. doi: 10.1016/s0278-2391(10)80466-5. PMID: 2341935
Kieser JA, van der Merwe CA
Arch Oral Biol 1984;29(10):795-801. doi: 10.1016/0003-9969(84)90009-8. PMID: 6594092

Prognosis

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
Filo K, Schneider T, Kruse AL, Locher M, Grätz KW, Lübbers HT
Swiss Dent J 2015;125(3):278-92. doi: 10.61872/sdj-2015-03-149. PMID: 26168686
Shah AJ, Kapoor N, Weinberg KI, Crooks GM, Kohn DB, Lenarsky C, Kaufman F, Epport K, Wilson K, Parkman R
Biol Blood Marrow Transplant 2002;8(4):221-8. doi: 10.1053/bbmt.2002.v8.pm12014811. PMID: 12014811
Sindet-Pedersen S, Enemark H
J Oral Maxillofac Surg 1990 Jun;48(6):554-8; discussion 559-60. doi: 10.1016/s0278-2391(10)80466-5. PMID: 2341935
Tulensalo T, Ranta R, Kataja M
Community Dent Oral Epidemiol 1989 Oct;17(5):258-62. doi: 10.1111/j.1600-0528.1989.tb00629.x. PMID: 2791517

Clinical prediction guides

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
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
Sindet-Pedersen S, Enemark H
J Oral Maxillofac Surg 1990 Jun;48(6):554-8; discussion 559-60. doi: 10.1016/s0278-2391(10)80466-5. PMID: 2341935
Tulensalo T, Ranta R, Kataja M
Community Dent Oral Epidemiol 1989 Oct;17(5):258-62. doi: 10.1111/j.1600-0528.1989.tb00629.x. PMID: 2791517
Kieser JA, van der Merwe CA
Arch Oral Biol 1984;29(10):795-801. doi: 10.1016/0003-9969(84)90009-8. PMID: 6594092

Recent systematic reviews

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
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
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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