Abstract

A case of pseudohypoparathyrodism has been presented, and the dental findings of pitting hypoplasia of the enamel of permanent teeth have been described. Ground sections indicated that the defect was primarily one of enamel formation and not ascribable to resorption of the fully formed enamel. Microradiographs demonstrated a calcification defect in the dentine, most conspicuous around the abnormal apices of the roots of teeth. A review of the literature indicates that evidence of the genetic transmission of PHP as a sex-linked dominant trait is incomplete. The sharing of clinical and/or chemical features by PHP, PPHP, the hypocalcemia-basal ganglia calcification syndrome, and the basal-cell nevi—odontogenic keratocysts of the jaw syndrome is discussed, and it is suggested that the tendency to develop medulloblastoma may be a related disorder.

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