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Oral and craniofacial manifestations of mucopolysaccharidoses
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Orientador(es)
Resumo(s)
Mucopolysaccharidoses (MPS) are a group of inherited metabolic disorders caused by the deficiency of
lysosomal enzymes necessary for the degradation of glycosaminoglycans (GAGs). Non-degraded GAGs
accumulate inside the lysosomes and compromise cell function in different tissues and organs. This
accumulation causes progressive and multisystemic damage, leading to a wide spectrum of clinical
manifestations, including oral and craniofacial manifestations. This work aims, therefore, to conduct a
literature review that promotes specific knowledge regarding the oral and craniofacial manifestations of
MPS. The results converged to a variability of alterations, among them facial dysmorphism,
macroglossia, lingual protrusion, anterior open bite, dental caries, gingival inflammation, enamel
hypoplasia, taurodontism, condylar hypoplasia and presence of dentigerouscysts. Preventive and
interceptive actions in oral health are used as a means of improving oral hygiene and reducing oral
problems. Finally, there is a need for improvement in comprehensive care for patients with MPS.
Descrição
Palavras-chave
Mucopolysaccharidoses Oral manifestations Craniofacial manifestations Oral health