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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.
See: Feature record | Search on this feature
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.
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

Professional guidelines

PubMed

Genotype-phenotype analysis of selective failure of tooth eruption-A systematic review.
Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
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
The influence of patients' decisions on treatment planning in restorative dentistry.
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

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
Effect of Tooth Agenesis on Mandibular Morphology and Position.
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
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
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

Diagnosis

Genotype-phenotype analysis of selective failure of tooth eruption-A systematic review.
Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
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
Prevalence and patterns of permanent tooth agenesis in individuals with Down syndrome: a meta-analysis.
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
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
Coeliac-type permanent-tooth enamel defects.
Aine L
Ann Med 1996 Feb;28(1):9-12. doi: 10.3109/07853899608999067. PMID: 8932499

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
Prosthetic replacement vs space closure for maxillary lateral incisor agenesis: A systematic review.
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
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
Prevalence of dental anomalies in nonsyndromic individuals with cleft lip and palate: a systematic review and meta-analysis.
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

The effect of endodontics and crown restoration techniques on the prognosis of treatment non-vital teeth.
Fastovets OO, Kotelevskyi RA, Matvyeyenko RY
Wiad Lek 2018;71(9):1738-1741. PMID: 30737932
Prevalence and patterns of permanent tooth agenesis in individuals with Down syndrome: a meta-analysis.
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
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
White-spot lesions during multibracket appliance treatment: A challenge for clinical excellence.
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

The Association of Developmental Dental Defects and the Clinical Consequences in the Primary Dentition: A Systematic Review of Observational Studies.
Portella PD, Dias BC, Ferreira P, de Souza JF, Wambier L, Assunção LRDS
Pediatr Dent 2022 Sep 15;44(5):330-341. PMID: 36309777
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
Prosthetic replacement vs space closure for maxillary lateral incisor agenesis: A systematic review.
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
Prevalence and patterns of tooth agenesis in Angle Class II Division 2 malocclusion in Japan.
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
Prevalence of dental anomalies in nonsyndromic individuals with cleft lip and palate: a systematic review and meta-analysis.
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

Recent systematic reviews

Genotype-phenotype analysis of selective failure of tooth eruption-A systematic review.
Guo X, Duan X
Clin Genet 2023 Sep;104(3):287-297. Epub 2023 Jul 13 doi: 10.1111/cge.14400. PMID: 37448157
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
Prosthetic replacement vs space closure for maxillary lateral incisor agenesis: A systematic review.
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
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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