Resumo

MEDEIROS, Annie Karoline Bezerra de; FARIAS, aCosta Rodrigues; PEREIRA, Hallissa Simplício Gomes  e  LIMA, Kenio Costa de. Berardinelli-Seip yndrome and oral health status: a literature review. RFO UPF [online]. 2014, vol.19, n.3, pp. 375-378. ISSN 1413-4012.

Objective: Dentistry involves the study of different aspects of the stomatognathic system, including peculiarities and rare conditions. Berardinelli-Seip syndrome (BSS) is a rare condition in which affected individuals exhibit metabolic abnormalities, a nearly complete absence of subcutaneous adipose tissue, heart problems, liver complications (such as hepatosplenomegaly) and accelerated bone growth. The aim of the present study was to perform a review of the literature on oral conditions associated with BSS. Materials and method: Searches were performed in the LILACS, MEDLINE, SciELO, PubMed and BBO databases using the descriptors "Berardinelli and carie", "Berardinelli and periodontal diseases" and "Berardinelli and malocclusion" to retrieve papers addressing the oral health status and dentofacial aspects of individuals with BSS. Case reports, case series, cross-sectional studies, clinical trials, cohort studies and case-control studies on this topic published in English, Spanish or Portuguese between 1954 and the present date were analyzed. Results: Three papers fulfilled the established inclusion criteria and revealed that individuals with BSS have a high rate of periodontal disease, such as significant gingivitis, deep periodontal pockets and clinical attachment loss. A high rate of tooth decay is also found, as well as malocclusions, such as anterior open bit, Angle skeletal classes I and II and severe crowding. Final considerations: Individuals with BSS exhibit a relatively poor oral health status.

Palavras-chave : Lipodystrophy; Congenital Generalized.; Oral health.; Malocclusion..