Open AccessAssociation of enamel-renal syndrome with sialolith: A rare entity
Author(s) -
Vengal Manoj,
N C Sandeepa,
M Selvamani,
Marish Panjami
Publication year - 2019
Publication title -
journal of oral and maxillofacial pathology/journal of oral and maxillofacial pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.455
H-Index - 25
eISSN - 1998-393X
pISSN - 0973-029X
DOI - 10.4103/jomfp.jomfp_9_18
Subject(s) - amelogenesis imperfecta , nephrocalcinosis , medicine , enamel paint , pathology , dermatology , dentistry , kidney
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.