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Enamel‐Renal‐Syndrome: case report
Author(s) -
Torres Luiz Henrique Soares,
deAzevedoVaz Sérgio Lins,
Barroso Danielle Resende Camisasca,
Silva Daniela Nascimento,
Velloso Tânia Regina Grão,
Barros Liliana Aparecida Pimenta
Publication year - 2018
Publication title -
special care in dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.328
H-Index - 41
eISSN - 1754-4505
pISSN - 0275-1879
DOI - 10.1111/scd.12288
Subject(s) - amelogenesis imperfecta , nephrocalcinosis , medicine , enamel paint , dentistry , connective tissue , pathology , kidney
Aims to describe a case in which dental changes were observed and investigation proceeded to consider Enamel‐Renal‐Syndrome (ERS), a rare disorder that associates amelogenesis imperfecta with nephrocalcinosis. Case report an 11‐year‐old male patient upon intraoral examination revealed generalized gingival hyperplasia, a few teeth were absent clinically and the remaining ones were yellowish‐brown in color. The enamel alterations were suggesting of amelogenesis imperfecta. Unerupted teeth with increased pericoronal spaces, suggesting hyperplasic follicles or dentigerous cysts and an enamel with lower thickness and density were observed in the panoramic radiography. The patient was referred for an assay to investigate mucopolysaccharidosis; however, it was negative. A renal ultrasound showed bilateral nephrocalcinosis and laboratory exams, including calcium, phosphate, and creatinine levels were below the average. An incisional gingival biopsy showed numerous round to ovoid basophilic calcifications in the connective tissue. The final diagnosis was ERS. Conclusion Dentists should refer patients with similar clinical presentation for renal ultrasound evaluation in order to rule out the possible diagnosis of ERS.