Abstract
BackgroundRare diseases (RD) are those that present a lower prevalence than 5 cases per 10.000 population. The main objective of this review was to study the effect on oral health in rare diseases, while the secondary objective of the study is theme upgrade.Material and MethodsComparative observational case-control studies were analysed and a systematic review was conducted in PubMed. Each rare disease listed on the statistical data record of the Health Portal of the Ministry of Equality, Health and Social Policies Board of Andalusia was associated with “oral health”. The variables studied included dental, oral mucosa and occlusion alterations, oral pathologies (caries, periodontal disease) and other alterations (mouth breathing, parafunctional habits, etc). A bias analysis of the variable caries was conducted.ResultsSix RD were selected through our inclusion and exclusion criteria (hypogammaglobulinemia, Rett syndrome, Marfan syndrome, Prader-Willi syndrome, cystic fibrosis and Cri du chat syndrome) in a total of 8 publications, of which four trials were classified as high risk of bias and one of them as medium risk. There were not trials with low risk of bias.ConclusionsThe main statistically significant differences found by Syndrome compared to a control group were in Hypogammaglobulinemia with a greater tendency to enamel hypoplasia and dry mouth. The Rett syndrome had, as well, a greater tendency to an anterior open bite, ogival palate, bruxism, mouth breathing and tongue thrusting. Prader-Willi syndrome had a tendency of dental erosion, and Cri du chat syndrome showed a higher association to Tannerella forsythia. Key words:Rare diseases, oral health.
Highlights
The European Commission defines rare diseases (RD) as all diseases with a prevalence lower than 5 cases per 10.000 population (1)
Rare diseases can occur at any age and they present a wide range of alterations and symptoms that vary from one disease to another, but from patient to patient according to the degree of affectation and evolution
We started the research on July 15th 2014 using publications from the Medline (PubMed) database; we typed the name of each RD from the list of the documented cases in Andalusia, except for Down syndrome (75), “AND” “oral health” in the search field
Summary
The European Commission defines rare diseases (RD) as all diseases with a prevalence lower than 5 cases per 10.000 population (1). Rare diseases can occur at any age and they present a wide range of alterations and symptoms that vary from one disease to another, but from patient to patient according to the degree of affectation and evolution. Manifestations, alterations and oral pathologies can be found within this diversity. The principal objective of this review was to study the affectation of oral health in RD, based on statistical data recorded in the Autonomous Community of Andalusia. The secondary objective is a theme upgrade
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