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Tooth agenesis, selective, 9(STHAG9)

MedGen UID:
934605
Concept ID:
C4310638
Congenital Abnormality
Synonym: STHAG9
 
Gene (location): GREM2 (1q43)
 
Monarch Initiative: MONDO:0014999
OMIM®: 617275

Definition

Selective tooth agenesis-9 (STHAG9) is an autosomal dominant disorder characterized by agenesis of various teeth. Other features include taurodontism, microdontia, short tooth roots, sparse and slow-growing hair, and dry and itchy skin (Kantaputra et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of selective tooth agenesis, see STHAG1 (106600). [from OMIM]

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.
Taurodontism
MedGen UID:
75596
Concept ID:
C0266039
Disease or Syndrome
Increased volume of dental pulp of permanent molar characterized by a crown body-root ratio equal or larger than 1:1 or an elongated pulp chambers and apical displacement of the bifurcation or trifurcation of the roots.
Selective tooth agenesis
MedGen UID:
370882
Concept ID:
C1970308
Congenital Abnormality
Agenesis specifically affecting one of the classes incisor, premolar, or molar.

Professional guidelines

PubMed

Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Kalsi JS, Hemmings K
Dent Update 2013 Nov;40(9):698-700, 702-4, 707-8, 710. doi: 10.12968/denu.2013.40.9.698. PMID: 24386761

Recent clinical studies

Etiology

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
Jurek A, Gozdowski D, Czochrowska EM, Zadurska M
Int J Environ Res Public Health 2021 Nov 12;18(22) doi: 10.3390/ijerph182211876. PMID: 34831629Free PMC Article
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
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Khalaf K, Miskelly J, Voge E, Macfarlane TV
J Orthod 2014 Dec;41(4):299-316. doi: 10.1179/1465313314Y.0000000116. PMID: 25404667

Diagnosis

Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
Palaska PK, Antonarakis GS
Eur J Oral Sci 2016 Aug;124(4):317-28. Epub 2016 Jun 17 doi: 10.1111/eos.12282. PMID: 27311636
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
Khalaf K, Miskelly J, Voge E, Macfarlane TV
J Orthod 2014 Dec;41(4):299-316. doi: 10.1179/1465313314Y.0000000116. PMID: 25404667
Aine L
Ann Med 1996 Feb;28(1):9-12. doi: 10.3109/07853899608999067. PMID: 8932499

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
Silveira GS, de Almeida NV, Pereira DM, Mattos CT, Mucha JN
Am J Orthod Dentofacial Orthop 2016 Aug;150(2):228-37. doi: 10.1016/j.ajodo.2016.01.018. PMID: 27476355
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
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
Tannure PN, Oliveira CA, Maia LC, Vieira AR, Granjeiro JM, Costa Mde C
Cleft Palate Craniofac J 2012 Mar;49(2):194-200. Epub 2011 Jul 8 doi: 10.1597/10-043. PMID: 21740173

Prognosis

Fastovets OO, Kotelevskyi RA, Matvyeyenko RY
Wiad Lek 2018;71(9):1738-1741. PMID: 30737932
Palaska PK, Antonarakis GS
Eur J Oral Sci 2016 Aug;124(4):317-28. Epub 2016 Jun 17 doi: 10.1111/eos.12282. PMID: 27311636
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
Hodder SL, Justman J, Hughes JP, Wang J, Haley DF, Adimora AA, Del Rio C, Golin CE, Kuo I, Rompalo A, Soto-Torres L, Mannheimer SB, Johnson-Lewis L, Eshleman SH, El-Sadr WM; HIV Prevention Trials Network 064; Women's HIV SeroIncidence Study Team
Ann Intern Med 2013 Jan 1;158(1):10-8. doi: 10.7326/0003-4819-158-1-201301010-00004. PMID: 23277896Free PMC Article
Enaia M, Bock N, Ruf S
Am J Orthod Dentofacial Orthop 2011 Jul;140(1):e17-24. doi: 10.1016/j.ajodo.2010.12.016. PMID: 21724067

Clinical prediction guides

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
Alqahtani ND, Alshammari R, Almoammar K, Almosa N, Almahdy A, Albarakati SF
Niger J Clin Pract 2019 Nov;22(11):1530-1538. doi: 10.4103/njcp.njcp_125_19. PMID: 31719274
Silveira GS, de Almeida NV, Pereira DM, Mattos CT, Mucha JN
Am J Orthod Dentofacial Orthop 2016 Aug;150(2):228-37. doi: 10.1016/j.ajodo.2016.01.018. PMID: 27476355
Ota K, Arai K
Am J Orthod Dentofacial Orthop 2015 Jul;148(1):123-9. doi: 10.1016/j.ajodo.2015.02.023. PMID: 26124035
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

Recent systematic reviews

Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
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
Silveira GS, de Almeida NV, Pereira DM, Mattos CT, Mucha JN
Am J Orthod Dentofacial Orthop 2016 Aug;150(2):228-37. doi: 10.1016/j.ajodo.2016.01.018. PMID: 27476355
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
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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