Atypical dentin dysplasia due to SMOC2 deficiency

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Synonyms: Dentin dysplasia type 1 with microdontia and shape anomalies

A rare genetic dentin dysplasia disease characterized by extreme microdontia oligodontia and abnormal tooth shape (including globular teeth incisal notches and double tooth formation). Short roots with a variable pulp phenotype (including taurodontia and flame-shaped pulp) enamel hypoplasia and anterior open bite may also be associated.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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Atypical dentin dysplasia due to SMOC2 deficiency?

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