Molar-incisor hypomineralization (MIH) is a condition that is defined based on its peculiar clinical presentation. Reports on the etiology of the condition and possible risk factors are inconclusive and the original suggestion that MIH is an idiopathic condition is often cited. Our group was the first to suggest MIH has a genetic component that involves genetic variation in genes expressed during dental enamel formation. In this report, we provide a rationale to explain the preferential affection of molars and incisors. We suggest that MIH is a genetic condition based on its prevalence, which varies depending on the geographic location, and the evidence that on occasion second primary molars, permanent canines, and premolars can show signs of hypomineralization of enamel when molars and incisors are affected.

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