Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family.

This is a report of a kindred of at least 181 members, of whom 35 exhibit or are reported to have dentin dysplasia type I. Six others are suspected of having the condition. Radiographic evidence that included obliterated or semilunar pulp chambers and short or undeveloped roots confirmed the diagnosis in 18 persons. The autosomal dominant mode of inheritance has been confirmed. One hundred percent penetrance has been demonstrated. There were insufficient data to determine the degree of expressivity.