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Spec Care Dentist. 2007 Mar-Apr;27(2):52-8.

Prevalence of oral diseases in Shwachman-Diamond syndrome.

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Department of Pediatric Dentistry, Faculty of Dentistry, University of Toronto, Ontario, Canada.


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.

[Indexed for MEDLINE]

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