Amelogenesis imperfecta with renal disease--a report of two cases.

Amelogenesis imperfecta (AI) is a collective term for a number of developmental conditions characterized by abnormal enamel formation. AI is usually not associated with generalized findings; however, a few cases of AI associated with syndromes and metabolic disorders have been reported in the literature. We report two cases of AI presenting with renal disease and thereby highlight the importance of recognizing this possible association at an early stage, as AI in some cases, may be a marker of renal disease.