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Original Research

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Amelogenesis imperfecta and nephrocalcinosis: a new case of this rare syndrome

  • Isabelle Normand de la Tranchade1,*,
  • Hervé Bonarek2
  • Jean-Marie Marteau3
  • Marie-José Boileau4
  • Javotte Nancy5

1 Université d'Odontologie de Bordeaux II, Service d'Odontologie pédiatrique, 7 rue Roquelaine, 31000 Toulouse, France and 16 cours de la Marne, 33000 Bordeaux, France

2Hopital Saint André, Service de Néphrologie, Cours d'Albret, 33000 Bordeaux, France

3 Université d'Odontologie de Bordeaux II, Service de Chirurgie Buccale, 16 cours de la Marne, 33000 Bordeaux, France

4Université d'Odontologie de Bordeaux II, Service d'Orthodontie, 16 cours de la Marne, 33000 Bordeaux, France

5 Université d'Odontologie de Bordeaux II, Service d'Odontologie Pédiatrique, 16 cours de la Marne, 33000 Bordeaux, France

DOI: 10.17796/jcpd.27.2.3188100w6t4516j8 Vol.27,Issue 2,April 2003 pp.171-176

Published: 01 April 2003

*Corresponding Author(s): Isabelle Normand de la Tranchade E-mail: i.normand@wanadoo.fr

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Abstract

This article describes a new case of a rare syndrome including enamel agenesis of the primary and permanent dentition, delayed or absent eruption of the permanent dentition, coronal intra-alveolar resorption and gingival enlargement. Renal symptoms include medullary nephrocalci-nosis without any apparent cause, and evolution to a renal failure. The early diagnosis provided by the oral symptoms leads to a better renal prognosis. As a consequence, pediatric dentists should be aware of this pathology.


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Isabelle Normand de la Tranchade,Hervé Bonarek,Jean-Marie Marteau,Marie-José Boileau,Javotte Nancy. Amelogenesis imperfecta and nephrocalcinosis: a new case of this rare syndrome. Journal of Clinical Pediatric Dentistry. 2003. 27(2);171-176.

URL:

https://www.jjocpd.com/articles/10.17796/jcpd.27.2.3188100w6t4516j8

References

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4. Lubinsky M, Angle C, Marsh PW, Witkop CJ. Syndrome of amelo-genesis imperfecta, nephrocalcinosis, impaired renal concentra-tion and possible abnormality of calcium metabolism. Am J Med Genet 20: 233-243, 1985

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8. Hall RK, Phakey P, Palamara J, Mc Credi DA. Amelogenesis imperfecta and nephrocalcinosis syndrome. Case studies of clini-cal features and ultrastructure of tooth enamel in two siblings. Oral Surg Oral Med Oral Pathol Oral Radiol End 79: 583-592, 1995.

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