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Association of enamel-renal syndrome with sialolith: A rare entity.


ABSTRACT: Amelogenesis imperfecta (AI) is a disease primarily affecting amelogenesis, but other various aberrations have been reported in association with this entity. Enamel-renal syndrome (ERS) is a very rare disorder associating AI with nephrocalcinosis. It is known by various synonyms such as AI nephrocalcinosis syndrome, MacGibbon syndrome, Lubinsky syndrome and Lubinsky-MacGibbon syndrome. The purposes of this article are to describe other dental anomalies which are rarely associated with AI making the diagnosis of AI a complex disorder and also to report a rare case of MacGibbon syndrome presented with sialolith of submandibular gland duct which is the first reported case in the literature.

SUBMITTER: Manoj V 

PROVIDER: S-EPMC6421903 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature

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Association of enamel-renal syndrome with sialolith: A rare entity.

Manoj V V   Sandeepa N C NC   Selvamani Manickam M   Panjami Marish M  

Journal of oral and maxillofacial pathology : JOMFP 20190201 Suppl 1


Amelogenesis imperfecta (AI) is a disease primarily affecting amelogenesis, but other various aberrations have been reported in association with this entity. Enamel-renal syndrome (ERS) is a very rare disorder associating AI with nephrocalcinosis. It is known by various synonyms such as AI nephrocalcinosis syndrome, MacGibbon syndrome, Lubinsky syndrome and Lubinsky-MacGibbon syndrome. The purposes of this article are to describe other dental anomalies which are rarely associated with AI making  ...[more]

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