Amelogenesis imperfecta among Israeli Jews and the description of a new type of local hypoplastic autosomal recessive amelogenesis imperfecta

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Abstract

Amelogenesis imperfecta (Al) was detected in ine of 70,359 school children surveyed, a prevalence approximating 1:8,000. Of these cases, eight were of the hypoplastic type and one the snow-capped hypomaturation type. Family studies demonstrated that hypoplastic Al was an autosomal dominant trait in two children and an autosomal recessive in six. Of three additional families referred to our clinic, two had autosomal recessive hypoplastic Al and one the hypocalcified type, inherited as an autosomal dominant trait. In four families, a new type of local hypoplastic autosomal recessive Al was observed, characterized by horizontal pitting and grooving more pronounced in the middle third of the crowns of most teeth in both dentitions.

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This investigation was supported in part by United States Public Health Service contract 06-663-1.

Department of Pedodontics.

∗∗

Department of Human Genetics, Faculty of Medicine.

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