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CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 35-39

Orodental manifestations of enamel renal syndrome in siblings: A rare case report


1 Department of Oral Medicine and Radiology, Subharti Dental College and Hospital, Meerut, Uttar Pradesh, India
2 Department of Oral Pathology and Microbiology, Subharti Dental College and Hospital, Meerut, Uttar Pradesh, India

Correspondence Address:
Jyoti Sharma
Department of Oral Medicine and Radiology, Subharti Dental College and Hospital, Meerut, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-6027.186665

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Amelogenesis imperfecta (AI) is a group of hereditary disorders that affect the quality and/or quantity of dental enamel. Hypoplastic AI can occur per se or in a variety of ectodermal syndromes and metabolic disorders. Enamel renal syndrome (ERS) is a very rare disorder associating hypoplastic AI with nephrocalcinosis. Since nephrocalcinosis is often asymptomatic, the orodental findings and dental radiographic features play a key role in the diagnosis of this extremely rare inherited disorder. This paper describes the orodental manifestations of ERS in siblings who were born of consanguineous parents.


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