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J Clin Pediatr Dent. 2003 Winter;27(2):171-5.

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

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Université d'Odontologie de Bordeaux II, Service d'Odontologie pédiatrique, 16 cours de la Marne, 33000 Bordeaux, France.


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 nephrocalcinosis 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.

[Indexed for MEDLINE]

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