Oral findings in patients with mucolipidosis type III

• Autor(a) principal: Cavalcante,Weber Céo
• Data de Publicação: 2012
• Outros Autores: Santos,Luciano Cincurá Silva, Santos,Josiane Nascimento dos, Vasconcellos,Sara Juliana de Abreu de, Azevedo,Roberto Almeida de, Santos,Jean Nunes dos
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Brazilian Dental Journal
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0103-64402012000400026
• topic: mucolipidosis; pseudo-Hurler's syndrome; lysosomal metabolic diseases; storage disease

Mucolipidosis type III is a rare, autosomal recessive disorder, which is part of a group of storage diseases as a result of inborn error of lysosomal enzyme metabolism. It is characterized by the gradual onset of signs and symptoms affecting the physical and mental development as well as visual changes, heart, skeletal and joint. Although oral findings associated with mucolipidosis type II have been extensively reported, there is a shortage of information on mucolipidosis type III. This paper presents radiological and histological findings of multiple radiolucent lesions associated with impacted teeth in the jaw of a 16 year-old youngster with mucolipidosis type III.