Abstract
A family with a pedigree displaying prolonged retention of deciduous dentition with accompanying retardation of eruption of the permanent dentition has been investigated. Delayed eruption was found to be distributed in this family in a pattern characteristic of an autosomal dominant trait. Additional findings in these families included extreme short stature, concave midfacial appearance, frontal bossing, delayed skeletal maturation, presence of Wormian bones in lambdoidal suture region of the skull, and large, open fontanelles in infancy.
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