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MeSH Review

Natal Teeth

 
 
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Disease relevance of Natal Teeth

 

High impact information on Natal Teeth

  • Those with the rarer Jackson-Lawler Syndrome (MIM #167210; PC-2) lack oral involvement but have natal teeth and cutaneous cysts [2].
  • Stratification of these data by keratin mutation confirmed the increased incidence of cyst formation and natal teeth among PC-2 patients, although cysts were more commonly seen in PC-1 than previously reported (25%-33%) [3].
  • Multiple natal teeth and oligodontia: a case report [4].
  • Hypodontia, hyperdontia, and natal teeth can be manifestations of the syndrome [5].
 

Gene context of Natal Teeth

  • In the Jackson-Lawler form (PC-2), pachyonychia is accompanied by multiple pilosebaceous cysts, natal teeth, and hair abnormalities [6].

References

  1. Syringomas, natal teeth and oligodontia: a new ectodermal dysplasia? Morrison, P.J., Young, I.D. Clin. Dysmorphol. (1996) [Pubmed]
  2. Mutation of a type II keratin gene (K6a) in pachyonychia congenita. Bowden, P.E., Haley, J.L., Kansky, A., Rothnagel, J.A., Jones, D.O., Turner, R.J. Nat. Genet. (1995) [Pubmed]
  3. Clinical and pathological features of pachyonychia congenita. Leachman, S.A., Kaspar, R.L., Fleckman, P., Florell, S.R., Smith, F.J., McLean, W.H., Lunny, D.P., Milstone, L.M., van Steensel, M.A., Munro, C.S., O'Toole, E.A., Celebi, J.T., Kansky, A., Lane, E.B. J. Investig. Dermatol. Symp. Proc. (2005) [Pubmed]
  4. Multiple natal teeth and oligodontia: a case report. Asquinazi, M.L., Pouezat, J.A., Jasmin, J.R. Refuʾat ha-peh ṿeha-shinayim (1993) (2001) [Pubmed]
  5. Oral aspects of Rubinstein-Taybi syndrome. Hennekam, R.C., Van Doorne, J.M. American journal of medical genetics. Supplement. (1990) [Pubmed]
  6. Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex. Smith, F.J., Corden, L.D., Rugg, E.L., Ratnavel, R., Leigh, I.M., Moss, C., Tidman, M.J., Hohl, D., Huber, M., Kunkeler, L., Munro, C.S., Lane, E.B., McLean, W.H. J. Invest. Dermatol. (1997) [Pubmed]
 
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