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Taurodontism, microdontia, and dens invaginatus(TMDI)

MedGen UID:
374171
Concept ID:
C1839235
Disease or Syndrome
Synonym: TMDI
 
Gene (location): KIF4A (Xq13.1)
 
Monarch Initiative: MONDO:0010740
OMIM®: 313490

Definition

Taurodontism, microdontia, and dens invaginatus (TMDI) is an X-linked recessive disorder in which affected individuals exhibit generalized abnormally small teeth, with molars that have a crown and pulp chamber that are disproportionately and vertically longer than the roots (taurodontism). In addition, multiple teeth exhibit dens invaginatus, the formation of a tooth within a tooth, resulting either from the dental papilla folding into the developing tooth or the enamel folding into the dental papilla (Casamassimo et al., 1978; Gowans et al., 2019). [from OMIM]

Recent clinical studies

Etiology

Wangsrimongkol T, Manosudprasit M, Pisek P, Chittiwatanapong N
J Med Assoc Thai 2013 Sep;96 Suppl 4:S25-35. PMID: 24386739
Gupta SK, Saxena P, Jain S, Jain D
J Oral Sci 2011 Jun;53(2):231-8. doi: 10.2334/josnusd.53.231. PMID: 21712629
Gulsahi A, Cebeci AI, Ozden S
Int Endod J 2009 Aug;42(8):735-9. Epub 2009 Jun 22 doi: 10.1111/j.1365-2591.2009.01580.x. PMID: 19549152
King NM, Tongkoom S, Itthagarun A, Wong HM, Lee CK
J Clin Pediatr Dent 2008 Winter;32(2):139-46. doi: 10.17796/jcpd.32.2.w76653r22rnnn713. PMID: 18389680
Ezoddini AF, Sheikhha MH, Ahmadi H
Community Dent Health 2007 Sep;24(3):140-4. PMID: 17958073

Diagnosis

Mehta V, Raheja A, Singh RK
BMJ Case Rep 2015 Dec 11;2015 doi: 10.1136/bcr-2015-211219. PMID: 26655667Free PMC Article
Wangsrimongkol T, Manosudprasit M, Pisek P, Chittiwatanapong N
J Med Assoc Thai 2013 Sep;96 Suppl 4:S25-35. PMID: 24386739
Gulsahi A, Cebeci AI, Ozden S
Int Endod J 2009 Aug;42(8):735-9. Epub 2009 Jun 22 doi: 10.1111/j.1365-2591.2009.01580.x. PMID: 19549152
Desai RS, Vanaki SS, Puranik RS, Rashmi GS, Nidawani P
J Oral Pathol Med 2006 Aug;35(7):407-9. doi: 10.1111/j.1600-0714.2006.00418.x. PMID: 16827843
Suprabha BS
J Indian Soc Pedod Prev Dent 2005 Sep;23(3):156-8. doi: 10.4103/0970-4388.16892. PMID: 16224139

Therapy

Gulsahi A, Cebeci AI, Ozden S
Int Endod J 2009 Aug;42(8):735-9. Epub 2009 Jun 22 doi: 10.1111/j.1365-2591.2009.01580.x. PMID: 19549152

Clinical prediction guides

Sobouti F, Dadgar S, Goleyjani P, Rakhshan V
BMC Oral Health 2023 Nov 17;23(1):877. doi: 10.1186/s12903-023-03504-y. PMID: 37978489Free PMC Article

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