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Tooth root resorption associated with a familial bone dysplasia affecting mother and daughter
C B Olsen1, K Tangchaitrong, I Chippendale
1Royal Dental Hospital of Melbourne/University of Melbourne, Australia.
Abstract:
The dental findings are presented of a mother and daughter who suffer from an as yet unclassified bone dysplasia that shows features of both hereditary hyperphosphatasia and familial expansile osteolysis. Both patients have experienced progressive root resorption of permanent teeth, deafness, and high alkaline phosphatase levels. The mother has a more advanced bone dysplasia which has led to progressive skeletal deformity and bone pain. The kindred is consistent with an autosomal dominant pattern, and the mutation(s) is thought to be in chromosome 18q21-22 region. Conventional treatment strategies of root resorption offer only a poor prognosis for the dentition. Therapy using alendronate, a bisphosphonate compound and a potent inhibitor of osteoclastic activity, has reduced alkaline phosphatase levels, bone pain, and may offer an effective strategy to prevent tooth root resorption in this group of diseases.
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