Berardinelli-Seip syndrome and oral health status: case series

Abstract

Background: Congenital Generalized Lipodystrophy, also known as Berardinelli-Seip syndrome (SBS), is a rare, autosomal recessive disease characterized by the nearly complete absence of subcutaneous adipose tissue beginning at birth. The metabolic, physiologic, biochemical and genetic characteristics of SBS are widely divulged among the scientific community. However, there are few studies on the possible oral conditions associated with this syndrome. Objective: the aim of the present study was to assess oral health status in a group of individuals with SBS and identify the occurrence of possible oral conditions. Methods: Evaluation of dental caries, treatment needs, degree of edentulism and periodontal health status using the methods recommended by the 2010 Oral Health Brazil project, in 10 people diagnosed with SBS in a Brazilian state. Results: The mean DMFT index was 16.7. The mean number of decayed teeth was 4.9. The mean number of missing teeth was 6.4. Regarding periodontal status, gingival bleeding was found in 61% of the sites examined, dental calculus was found in 35% and shallow pockets were found in six of the 54 sites evaluated. Conclusions: The prevalence of gingival bleeding was high, demonstrating gingivitis among the participants. Was an absence of cases with advanced periodontal disease. The participants had a low degree of edentulism, but high DMFT index values.