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Tooth agenesis, selective, 7(STHAG7)

MedGen UID:
899184
•Concept ID:
C4225231
•
Disease or Syndrome
Synonym: STHAG7
 
Gene (location): LRP6 (12p13.2)
 
Monarch Initiative: MONDO:0014749
OMIM®: 616724

Definition

Any tooth agenesis in which the cause of the disease is a mutation in the LRP6 gene. [from MONDO]

Clinical features

From HPO
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
Agenesis of permanent teeth
MedGen UID:
224851
•Concept ID:
C1290511
•
Congenital Abnormality
A congenital defect characterized by the absence of one or more permanent teeth, including oligodontia, hypodontia, and adontia of the of permanent teeth.
See: Feature record | Search on this feature

Professional guidelines

PubMed

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
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

Recent clinical studies

Etiology

Variant characterisation and clinical profile in a large cohort of patients with Ellis-van Creveld syndrome and a family with Weyers acrofacial dysostosis.
Altunoglu U, Palencia-Campos A, Güneş N, Turgut GT, Nevado J, Lapunzina P, Valencia M, Iturrate A, Otaify G, Elhossini R, Ashour A, K Amin A, Elnahas RF, Fernandez-Nuñez E, Flores CL, Arias P, Tenorio J, Chamorro Fernández CI, Güven Y, Özsu E, Eklioğlu BS, Ibarra-Ramirez M, Diness BR, Burnyte B, Ajmi H, Yüksel Z, Yıldırım R, Ünal E, Abdalla E, Aglan M, Kayserili H, Tuysuz B, Ruiz-Pérez V
J Med Genet 2024 Jun 20;61(7):633-644. doi: 10.1136/jmg-2023-109546. PMID: 38531627
Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Morphologic and Demographic Predictors of Third Molar Agenesis: A Systematic Review and Meta-analysis.
Carter K, Worthington S
J Dent Res 2015 Jul;94(7):886-94. Epub 2015 Apr 16 doi: 10.1177/0022034515581644. PMID: 25883107
A controlled study of associated dental anomalies.
Baccetti T
Angle Orthod 1998 Jun;68(3):267-74. doi: 10.1043/0003-3219(1998)068<0267:ACSOAD>2.3.CO;2. PMID: 9622764
Hypodontia, hyperodontia, and double formation of primary teeth in Iceland. An epidemiological study.
Magnússon TE
Acta Odontol Scand 1984 Jun;42(3):137-9. doi: 10.3109/00016358408993864. PMID: 6332449

Diagnosis

Variant characterisation and clinical profile in a large cohort of patients with Ellis-van Creveld syndrome and a family with Weyers acrofacial dysostosis.
Altunoglu U, Palencia-Campos A, Güneş N, Turgut GT, Nevado J, Lapunzina P, Valencia M, Iturrate A, Otaify G, Elhossini R, Ashour A, K Amin A, Elnahas RF, Fernandez-Nuñez E, Flores CL, Arias P, Tenorio J, Chamorro Fernández CI, Güven Y, Özsu E, Eklioğlu BS, Ibarra-Ramirez M, Diness BR, Burnyte B, Ajmi H, Yüksel Z, Yıldırım R, Ünal E, Abdalla E, Aglan M, Kayserili H, Tuysuz B, Ruiz-Pérez V
J Med Genet 2024 Jun 20;61(7):633-644. doi: 10.1136/jmg-2023-109546. PMID: 38531627
Prevalence of Premature Eruption and Agenesis of Premolars in Turkish Children: A Retrospective Study.
Koc N, Ballikaya E, Cehreli ZC
J Clin Pediatr Dent 2021 Jan 1;45(1):58-62. doi: 10.17796/1053-4625-45.1.11. PMID: 33690831
Diagnostic and severity scores for Cockayne syndrome.
Spitz MA, Severac F, Obringer C, Baer S, Le May N, Calmels N, Laugel V
Orphanet J Rare Dis 2021 Feb 3;16(1):63. doi: 10.1186/s13023-021-01686-8. PMID: 33536051Free PMC Article
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
The genetic basis of inherited anomalies of the teeth. Part 2: syndromes with significant dental involvement.
Bailleul-Forestier I, Berdal A, Vinckier F, de Ravel T, Fryns JP, Verloes A
Eur J Med Genet 2008 Sep-Oct;51(5):383-408. Epub 2008 May 23 doi: 10.1016/j.ejmg.2008.05.003. PMID: 18599376

Therapy

Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Comparison of root and canal anatomy of taurodont and normal molar teeth: A retrospective cone-beam computed tomography study.
Aydın H, Mobaraki S
Arch Oral Biol 2021 Oct;130:105242. Epub 2021 Aug 13 doi: 10.1016/j.archoralbio.2021.105242. PMID: 34411883
Inferior alveolar nerve block and third-molar agenesis: a retrospective clinical study.
Swee J, Silvestri AR Jr, Finkelman MD, Rich AP, Alexander SA, Loo CY
J Am Dent Assoc 2013 Apr;144(4):389-95. doi: 10.14219/jada.archive.2013.0132. PMID: 23543693
Surgical complications of segmental Le Fort I osteotomy.
Ho MW, Boyle MA, Cooper JC, Dodd MD, Richardson D
Br J Oral Maxillofac Surg 2011 Oct;49(7):562-6. Epub 2010 Oct 20 doi: 10.1016/j.bjoms.2010.09.018. PMID: 20965624
An epidemiological survey of occlusal anomalies among secondary school children in Ibadan, Nigeria.
Onyeaso CO
Odontostomatol Trop 2003 Jun;26(102):25-9. PMID: 14560680

Prognosis

A Case Against the Implant.
Robbins JW
Int J Periodontics Restorative Dent 2024 May 24;44(3):250-251. doi: 10.11607/prd.2024.3.e. PMID: 38787712
Diagnostic and severity scores for Cockayne syndrome.
Spitz MA, Severac F, Obringer C, Baer S, Le May N, Calmels N, Laugel V
Orphanet J Rare Dis 2021 Feb 3;16(1):63. doi: 10.1186/s13023-021-01686-8. PMID: 33536051Free PMC Article
Morphologic and Demographic Predictors of Third Molar Agenesis: A Systematic Review and Meta-analysis.
Carter K, Worthington S
J Dent Res 2015 Jul;94(7):886-94. Epub 2015 Apr 16 doi: 10.1177/0022034515581644. PMID: 25883107
Inferior alveolar nerve block and third-molar agenesis: a retrospective clinical study.
Swee J, Silvestri AR Jr, Finkelman MD, Rich AP, Alexander SA, Loo CY
J Am Dent Assoc 2013 Apr;144(4):389-95. doi: 10.14219/jada.archive.2013.0132. PMID: 23543693
Total mandibular alveolar osteotomy.
Rittersma J
J Oral Surg 1981 Nov;39(11):903-6. PMID: 6945417

Clinical prediction guides

A Case Against the Implant.
Robbins JW
Int J Periodontics Restorative Dent 2024 May 24;44(3):250-251. doi: 10.11607/prd.2024.3.e. PMID: 38787712
Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Diagnostic and severity scores for Cockayne syndrome.
Spitz MA, Severac F, Obringer C, Baer S, Le May N, Calmels N, Laugel V
Orphanet J Rare Dis 2021 Feb 3;16(1):63. doi: 10.1186/s13023-021-01686-8. PMID: 33536051Free PMC Article
Morphologic and Demographic Predictors of Third Molar Agenesis: A Systematic Review and Meta-analysis.
Carter K, Worthington S
J Dent Res 2015 Jul;94(7):886-94. Epub 2015 Apr 16 doi: 10.1177/0022034515581644. PMID: 25883107
Total mandibular alveolar osteotomy.
Rittersma J
J Oral Surg 1981 Nov;39(11):903-6. PMID: 6945417

Recent systematic reviews

Self-correction of the ectopic eruption of the maxillary first permanent molar and its predictive factors: A systematic review.
Bastos RTDRM, Dos Santos CCO, Bellini-Pereira SA, Normando D
Int J Paediatr Dent 2024 Jul;34(4):348-359. Epub 2023 Oct 24 doi: 10.1111/ipd.13131. PMID: 37876132
Tooth abnormalities associated with non-syndromic cleft lip and palate: systematic review and meta-analysis.
Fonseca-Souza G, de Oliveira LB, Wambier LM, Scariot R, Feltrin-Souza J
Clin Oral Investig 2022 Aug;26(8):5089-5103. Epub 2022 Jun 21 doi: 10.1007/s00784-022-04540-8. PMID: 35729285
Morphologic and Demographic Predictors of Third Molar Agenesis: A Systematic Review and Meta-analysis.
Carter K, Worthington S
J Dent Res 2015 Jul;94(7):886-94. Epub 2015 Apr 16 doi: 10.1177/0022034515581644. PMID: 25883107
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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