Abstract
The association of taurodontism with hypoplastic/hypomature enamel defects is presented in 2 cases of X-chromosome aneuploidy (47,XXY) [Klinefelter''s syndrome] and 1 of X-linked recessive amelogenesis imperfecta. The X-chromosome apparently plays a role in tooth size (the degree of taurodontism increasing with increased number of X chrosomes), and in enamel maturation.

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