title: Prevalence of oral diseases in Shwachman-Diamond syndrome.
abstract: The aim of this study was to determine the prevalence and severity of oral diseases in patients with ShwachmanDiamond syndrome (SDS). Thirty-five persons with SDS were compared to 20 healthy controls. A cross-sectional survey was carried out using self-reporting questionnaires and dental radiographs collected from the subjects and their dentists. Overall, oral diseases were more prevalent among subjects with SDS when compared to controls (p < 0.001). Persons with SDS also had more caries in both primary (p < 0.03) and permanent dentitions (p < 0.01), and also had delayed dental development (p < 0.04). Oral soft tissue pathoses, such as recurrent oral ulcerations (p < 0.00) and gingival bleeding upon brushing (p < 0.00), were significantly more prevalent in subjects with SDS. Pain on eating was also more frequent amongst persons with SDS (p < 0.008) and was often associated with oral ulcerations (p < 0.002). In conclusion, based on self-completed subject and dentist questionnaires, diseases of oral hard and soft tissues were more prevalent and severe in persons with SDS when compared with healthy controls.
authors.affiliation: Department of Pediatric Dentistry, Faculty of Dentistry, University of Toronto, Ontario, Canada.
authors.forename: William
authors.initials: W
authors.lastname: Ho
authors.forename: Chrisovalantou
authors.initials: C
authors.lastname: Cheretakis
authors.forename: Peter
authors.initials: P
authors.lastname: Durie
authors.forename: Gajanan
authors.initials: G
authors.lastname: Kulkarni
authors.forename: Michael
authors.initials: M
authors.lastname: Glogauer
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003731:Dental Caries / Q000453:epidemiology* / Q000209:etiology; D004812:Epidemiologic Methods; D005260:Female; D005891:Gingivitis / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000150:complications* / Q000151:congenital / Q000628:therapy; D019226:Oral Ulcer / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology; D010182:Pancreatic Diseases / Q000150:complications* / Q000628:therapy; D013577:Syndrome; D014071:Tooth Abnormalities / Q000453:epidemiology / Q000209:etiology