Oral Health in PraderWilli Syndrome Andrei Barasch DMD

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

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

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

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

Candidiasis

Gingivitis

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

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