Resumo

HERNANDEZ CANCINO, Claudia Marcela; NEMOTO VERGARA SASADA, Isabel; FISCHINGER MOURA DE SOUZA, Carolina  e  OLIVEIRA, Marieli. Mucopolysaccharidosis: characteristics and oral changes. RFO UPF [online]. 2016, vol.21, n.3, pp. 395-400. ISSN 1413-4012.

Objective: to perform a literature review on mucopolysaccharidosis addressing systemic characteristics associated with oral features, thus allowing a proper dental treatment and a better quality of life of the patient. Literature review: mucopolysaccharidosis is an inherited metabolic disorder caused by innate errors of metabolism, which cause the deficiency of lysosomal enzymes that degrade glycosaminoglycans and cause their accumulation within different tissues and organs. This abnormal accumulation compromises cellular and organic functions, leading to a great number of progressive and multisystemic clinical manifestations. Oral manifestations vary according to the type of mucopolysaccharidosis and are of major importance. Most individuals present large mouth, prominent lips, macroglossia, open anterior bite, small and wide mandible, limited mouth opening, gingival hyperplasia, mouth breathing, dental impaction, enamel hypoplasia, diastema with relative microdontia, taurodontism, hyperplasia of dental follicles, and dentigerous cysts. Follow-up should be continuous and multidisciplinary, monitoring patient evolution, foreseeing potential complications, and correcting rising dysfunctions, as well as evaluating therapy effectiveness. Final considerations: there are countless oral manifestations, so the professional is required to know mucopolysaccharidoses and monitor patients from childhood to adulthood, aiming to offer prevention, oral health maintenance, and specialized and multidisciplinary care.

Palavras-chave : Mucopolysaccharidosis; Pathology; Oral health.