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Oral Health in Prader-Willi Syndrome Andrei Barasch, DMD, MDSc Chair, Dept. of Dental Medicine Winthrop University Hospital

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Oral Changes in PWS PWS is a multi-systemic genetic disorder (chromosome 15) Oral physiology and pathology not adequately studied The largest study included only 50 subjects aged 5 to 40 years (Saeves et al, Int J Ped Dent 2012) Majority of publications are case reports

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Salivary Abnormalities in PWS Decreased salivary flow has been the only consistently documented oral finding Thick, sticky or frothy saliva is common Amount produced equivalent to about 1/3 to 1/5 normal Results in decreased cleansing, taste perception, remineralization, immune function

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Other reported oral findings Mucosa: trauma due to picking; candidiasis Dentition: hypodontia; enamel defects; rampant caries Gums: periodontal disease

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Candidiasis

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Gingivitis

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Potential Solutions Impeccable hygiene from the very start Frequent dental appointments Application of preventive measures (e.g. sealants, floride) Avoid refined carbohydrates Avoid lemon drops, even if sugarless Avoid acidic foods and drinks Rx: sialogogues and antimicrobial rinses

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The future We need more study to better define problems and possible solutions Potential identification of the genetic mechanism Potential targeted therapy