Prevalence of oral diseases in Shwachman-Diamond syndrome.

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.