1. 12 patients diagnosed as mucopolysaccharidosis (Hurler syndrome-3 cases, Scheie syndrome-1 case, Hunter syndrome-3 cases, Sanfilippo syndrome-2 cases, Morquio syndrome-3 cases) and 2 patients diagnosed as mucolipidosis (I-cell disease) were reviewed radiographically.
2. The localized dentigerous cyst-like bone changes and hypoplastic condyles in jaws were defined as two major radiographic key signs in mucopolysaccharidosis and I-cell disease.
3. Through published literatures and our radiograms, the localized dentigerous cystlike bone changes were probably related closely to the storage of mucopolysaccharide, and abnormal hypoplastic condyles were possibly caused by the unbalance of bone metabolism in mandibular ossification.
4. The radiographic findings of enlarged pulp chamber were found to be a new sign in affected teeth.
5. Mucopolysaccharidosis might be subsequently detected following minor X-ray findings, i. e., spacing of teeth, mandibular prognathism and underdevelopment of maxilla, etc, and with together two major key signs described above.
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