Catalogo · 9780494072318
Characterization of oral diseases in Shwachman-Diamond syndrome
Shwachman-Diamond syndrome (SDS) is an autosomal recessive disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction and skeletal abnormalities. Oral health status of SDS patients has never been investigated. Objectives of this project were (1) to determine the prevalence and severity of oral diseases in SDS patients and (2) to determine if oral diseases contribute to morbidity in SDS. Cross sectional mail survey questionnaires were completed by SDS patients, their parents, healthy siblings (controls) and dentists. Dental radiographs were also collected. Differences in oral findings were compared with the use of Student's t-tests and chi2 analyses. 55 subjects (35 SDS) participated in the study. Oral pathoses such as dental caries, delayed dental development, recurrent oral ulcerations and gingival bleeding were significantly more prevalent and severe among SDS patients than controls. Pain on eating contributes to morbidity in SDS patients.
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