상아질 형성 부전증

Abstract

Based on three cases(two autopsies and one biopsy) we have conducted a pathological study On dentino genesis imperfecta. Two cases were classified into type I dentinogenesis imperfecta, which were associated with congenital osteogenesis imperfecta. They were found in a newborn and a stillborn infan t. The lesions were characterized by irregular deposition of fibrillar dentine matrix, disturbed odontoblast layer and invagination of odontoblasts, reduction of dentinal tubules, and poor calcification of dentine. One case was classified into type II dentinogenesis imperfecta. This was a 25 years old male whose family showed a strong hereditary tendency of autosomal dominant character through four generations. Their teeth showed dark brown or blue discoloration, easy fragility, severe attrition and microdontism. Extracted teeth for full denture construction were examined histologically, and they showed marked reduction and atrophy of dentinal tubules, obliteration of pulp chamber and immature dentine formation