Impaired oral health in patients with aspartylglucosaminuria

• Published in: Oral surgery, oral medicine, oral pathology, oral radiology and endodontics oral medicine, oral pathology, oral radiology and endodontics, 1998-11, Vol.86 (5), p.562-568
• Main Authors: Arvio, Pekka, Arvio, Maria, Wolf, Juhani, Lukinmaa, Pirjo-Liisa, Saxen, Leena, Pirinen, Sinikka
• Format: Article
• Language: English
• Subjects: Acetylglucosamine - urine; Adolescent; Adult; Aged; Alveolar Bone Loss - diagnostic imaging; Aspartylglucosaminuria; Biological and medical sciences; Case-Control Studies; Child; Child, Preschool; Dental Caries - etiology; Dentistry; DMF Index; Facial bones, jaws, teeth, parodontium: diseases, semeiology; Female; Finland; Humans; Lysosomal Storage Diseases - complications; Male; Medical sciences; Middle Aged; Mouth Diseases - etiology; Mouth Neoplasms - etiology; Non tumoral diseases; Odontogenic Tumors - etiology; Oral Hygiene Index; Otorhinolaryngology. Stomatology; Periodontal Diseases - etiology; Periodontal Index; Radiography; Tooth Loss - etiology
• ISSN: 1079-2104; 1528-395X
• DOI: 10.1016/S1079-2104(98)90346-8

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. Study design. 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.