IMPAIRED ORAL HEALTH IN PATIENTS WITH ASPARTYLGLUCOSAMINURIA

Objective. The aim of this study was to assess the oral health of pati ents with aspartylglucosaminuria, a heritable lysosomal storage disord er, and to recommend guidelines for treatment. Study design. Eighty-tw o patients with aspartylglucosaminuria and 122 control subjects were e xamined clinically; in addition, panoramic radiographs were evaluated in 61 patients with aspartylglucosaminuria and 61 control subjects. Re sults. High prevalences of caries, gingivitis, and oral Candida (P < . 001), extensive gingival overgrowths (18%; P < .001), benign odontogen ic tumors or tumorlike lesions (8%; P = .057), reduced maxillary sinus es (P < .001), limited mouth opening (P < .001), and food retention in the mouth (45%) were the major oral findings that distinguished the p atients with aspartylglucosaminuria from the control subjects. Adults with aspartylglucosaminuria had diverse oral health problems, early lo ss of several permanent teeth being the most disabling feature. Conclu sions. Patients with aspartylglucosaminuria appear to be at a higher r isk for a number of oral disorders; however, poor oral hygiene and fai lure to cooperate increase these patients' risk of dental and periodon tal diseases, making successful prevention crucial.