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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
Goel S, Sahu S, Minz RW, Singh S, Suri D, Oh YM, Rawat A, Sehgal S, Saikia B
Front Immunol 2018;9:1080. Epub 2018 May 17 doi: 10.3389/fimmu.2018.01080. PMID: 29868029Free PMC Article
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Maruko E, Hayes C, Evans CA, Padwa B, Mulliken JB
Cleft Palate Craniofac J 2001 Jan;38(1):15-9. doi: 10.1597/1545-1569_2001_038_0015_hihm_2.0.co_2. PMID: 11204676

Diagnosis

Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
Goel S, Sahu S, Minz RW, Singh S, Suri D, Oh YM, Rawat A, Sehgal S, Saikia B
Front Immunol 2018;9:1080. Epub 2018 May 17 doi: 10.3389/fimmu.2018.01080. PMID: 29868029Free PMC Article
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
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

Portella PD, Dias BC, Ferreira P, de Souza JF, Wambier L, Assunção LRDS
Pediatr Dent 2022 Sep 15;44(5):330-341. PMID: 36309777
Kiliaridis S, Sidira M, Kirmanidou Y, Michalakis K
Eur J Oral Implantol 2016;9 Suppl 1:S5-24. PMID: 27314109
Pillai KG, Scipio JE, Nayar K, Louis N
West Indian Med J 2007 Sep;56(4):368-71. PMID: 18198744
Onyeaso CO, Oneyeaso AO
Oral Health Prev Dent 2006;4(3):181-6. PMID: 16961026
Magnússon TE
Community Dent Oral Epidemiol 1977 Nov;5(6):292-300. doi: 10.1111/j.1600-0528.1977.tb01017.x. PMID: 272256

Prognosis

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
Fastovets OO, Kotelevskyi RA, Matvyeyenko RY
Wiad Lek 2018;71(9):1738-1741. PMID: 30737932
Goel S, Sahu S, Minz RW, Singh S, Suri D, Oh YM, Rawat A, Sehgal S, Saikia B
Front Immunol 2018;9:1080. Epub 2018 May 17 doi: 10.3389/fimmu.2018.01080. PMID: 29868029Free PMC Article
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
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

Clinical prediction guides

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
Portella PD, Dias BC, Ferreira P, de Souza JF, Wambier L, Assunção LRDS
Pediatr Dent 2022 Sep 15;44(5):330-341. PMID: 36309777
Goel S, Sahu S, Minz RW, Singh S, Suri D, Oh YM, Rawat A, Sehgal S, Saikia B
Front Immunol 2018;9:1080. Epub 2018 May 17 doi: 10.3389/fimmu.2018.01080. PMID: 29868029Free PMC Article
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
Pillai KG, Scipio JE, Nayar K, Louis N
West Indian Med J 2007 Sep;56(4):368-71. PMID: 18198744

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
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
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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