Hereditary opalescent dentin: a report of two cases.

[dentinogenesis imperfecta]

The aim of this case report is to present the clinical and radiographic findings of hereditary opalescent dentin to facilitate an early diagnosis .Hereditary opalescent dentin (or dentinogenesis imperfecta ) may manifest itself in three variations : i .e ., Shields type I , Shields type II , and Shields type III . Dentinogenesis imperfecta occurs as an autosomal dominant trait with variable expressivity , either in presence with osteogenesis imperfecta or as a separate clinical entity in persons who have none of the features of osteogenesis imperfecta .A seven -year old boy and his mother were both diagnosed with hereditary opalescent dentin . A review of the family dental history revealed that this condition affected not only the child 's mother but his maternal grandfather and great grandfather . Both the son and the mother exhibited the same clinical and radiologic features as those reported previously with no evidence of osteogenesis imperfecta .Being an autosomal disease , hereditary opalescent dentin runs in the family and can affect both the deciduous and permanent dentitions as a dominant trait .Once a patient is diagnosed with hereditary opalescent dentin , other family members should be evaluated given the condition is hereditary .

Diseases
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Diseases presenting "maternal grandfather" symptom

dentinogenesis imperfecta

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