Oral health in Prader-Willi syndrome (completed)
About the project
Prader-Willi syndrome is rare and caused by a mutation on chromosome 15. Many tissues and organs are affected, and also the oral cavity. Hypotonic orofacial muscles, high and narrow palate, little and sticky saliva, increased caries activity and excessive tooth wear have been described.
The aim of the study is to increase the knowledge of oral health in PWS. Individuals with PWS (n=50) and matched controls (n=50) have been included and all clinical examinations have been performed. Oral examinations have focused on the prevalence of dental caries, tooth wear and gingival inflammation. Orofacial function have been analysed and salivary secretory rates measured. After analysing a few salivary samples by MALDI-TOF salivary cystatines were found to be possibly upregulated in PWS and are further analysed by immunohistichemic methods. Also salivary mucins are analysed. Impressions have been taken in order to make dental casts for evaluation. Exfoliated and extracted teeth are being examined by electron and light microscopy and microradiography to explore the enamel structure. Three international publications are planned. One publication has been submitted. Some laboratory and statistical analyses remain before the last publications can be written. Clinical studies on a group of individuals with development delay are demanding. The aim of the project is to increase the knowledge of oral health in PWS. This is necessary in order to provide the best possible care for this group of individuals.