Oral Health in PWS

This blog was coauthored by Jessica Bohonowych and Theresa Strong

Amongst the various health issues associated with PWS, dental concerns are some of the least well studied and understood. Overall dental health can have huge impacts on susceptibility to other diseases and plays an important role in overall quality of life, well beyond basic cosmetic concerns.

Several reports have described altered oral health in PWS patients including reduced enamel, increased frequency of caries (cavities), and thick or viscous saliva (Bazopoulou-Kyrkanidou 1992, Bailleul-Forestier 2008, Hart 1998). Importantly, two recent papers now also highlight increased tooth wear and a risk of early onset periodontitis in PWS.

Saeves (2012) (note: the full paper can be downloaded for free) report significantly more tooth wear in a study of 49 PWS patients as compared to an age and sex matched control group (age 6-40). Although unable to confirm a specific cause, the authors discuss several associated factors that correlate and may contribute to increased tooth wear in PWS. These include a greater frequency of tooth grinding as well as decreased saliva production, also referred to as hyposalivation. Teeth grinding, or bruxism, was reported in 18 versus 3 participants for PWS and control groups, respectively. Hyposalivation was observed in more than 50% of the PWS group as compared to just 3% of the control group. Saliva plays an important role in oral health, serving as a buffer to protect enamel against acidic foods and liquids, as well as a lubricant during chewing, similar to a well oiled engine.

A second report (Yanagita 2012) documents a case study of a 20-year-old PWS patient with severe periodontitis. Periodontitis is a severe inflammation of the supporting tissues surrounding teeth, including bones and gums. Left untreated, periodontitis usually leads to loosening, and ultimately loss, of teeth. Because periodontitis is a progressive disease, resulting from the increased worsening and development of gingivitis over time, it is uncommon throughout childhood and young adulthood. It is therefore important to understand what factors may contribute to an increased risk of early onset periodontitis in PWS so as to be able to take aggressive proactive preventative measures.

Although dental problems are common in PWS, a specific underlying genetic component has not been identified. There is some evidence that altered immunity, specifically immune hyperactivity and chronic inflammation, may be a contributing factor (Viardot 2010). The good news is that recent studies out of Europe suggest that early intervention and education, along with a controlled diet and strong oral hygiene are successful preventative measures with respect to oral health in PWS. In Norway, a case controlled study of 50 age and sex-matched controls shows that despite significantly decreased saliva production, the PWS group did not have more cavities or tooth decay relative to control (Saeves 2012). In addition, a survey of 15 PWS patients shows an improvement of overall oral health as compared to previous studies and attributes this to early diet management and strong oral hygiene (Bailleul-Forestier 2008).

Prevention is key as patients are more likely to learn and successfully execute preventative measures rather than the extensive treatments with complex post-operative self care needed in cases of significant dental problems. In addition, treatments once conditions have developed can be time consuming and painful. Preventative measures may include: setting good habits early in childhood; guidance on appropriate brushing technique from dental professionals to control plaque; regular visits to the dentist for cleaning, increasing water consumption (always a challenge in PWS!) over acidic liquids such as soft drinks to protect enamel; monitoring of tooth grinding; use of over the counter ‘dry mouth’ products such as Biotene; use of a mouth guard; and orthondtia to improve bite and teeth malposition, which may contribute to tooth wear. Looking ahead, new technologies such as cell (Nelson 2012) and gene therapy (Baum 2009) are being explored to permanently restore normal function in cases where salivary glands are not adequately hydrating the mouth.

PWS global registry

Topics: Research

Jessica Bohonowych

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Jessica Bohonowych is a graduate of Duke University, and holds a PhD in Pharmacology and Toxicology at the University of California, Davis. Incorporating her research background, knowledge of pharmacology and drug development, and teaching experience, Jessica works with Theresa Strong in managing FPWR’s grant portfolio, communicating research results and breakthroughs to our community, aiding in special projects such as the Clinical Trials Initiative and Molecular Resource Center, and is heading the development of the Global PWS Registry.

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