Impaired oral health in patients with aspartylglucosaminuria.
OBJECTIVE
The aim of this study was to assess the oral health of patients with aspartylglucosaminuria, a heritable lysosomal storage disorder, and to recommend guidelines for treatment.


METHODS
Eighty-two patients with aspartylglucosaminuria and 122 control subjects were examined clinically; in addition, panoramic radiographs were evaluated in 61 patients with aspartylglucosaminuria and 61 control subjects.


RESULTS
High prevalences of caries, gingivitis, and oral Candida (P < .001), extensive gingival overgrowths (18%; P < .001), benign odontogenic tumors or tumorlike lesions (8%; P = .057), reduced maxillary sinuses (P < .001), limited mouth opening (P < .001), and food retention in the mouth (45%) were the major oral findings that distinguished the patients with aspartylglucosaminuria from the control subjects. Adults with aspartylglucosaminuria had diverse oral health problems, early loss of several permanent teeth being the most disabling feature.


CONCLUSIONS
Patients with aspartylglucosaminuria appear to be at a higher risk for a number of oral disorders; however, poor oral hygiene and failure to cooperate increase these patients' risk of dental and periodontal diseases, making successful prevention crucial.
Arvio
P
P
Lammi Health Care Centre, Helsinki, Finland.
Arvio
M
M
Wolf
J
J
Lukinmaa
P L
PL
Saxen
L
L
Pirinen
S
S
D000117:Acetylglucosamine / Q000652:urine; D000293:Adolescent; D000328:Adult; D000368:Aged; D016301:Alveolar Bone Loss / Q000000981:diagnostic imaging; D054880:Aspartylglucosaminuria; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004245:DMF Index; D003731:Dental Caries / Q000209:etiology; D005260:Female; D005387:Finland; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000209:etiology*; D009062:Mouth Neoplasms / Q000209:etiology; D009808:Odontogenic Tumors / Q000209:etiology; D009911:Oral Hygiene Index; D010510:Periodontal Diseases / Q000209:etiology; D010512:Periodontal Index; D011859:Radiography; D016388:Tooth Loss / Q000209:etiology
D000117:Acetylglucosamine