Manifestaciones orales del síndrome de Maroteaux-Lamy (Mucopolisacaridosis VI)

Abstract

Mucopolysaccharidosis type VI, also known as Maroteaux-Lamy syndrome, is an autosomal recessive lysosomal disorder, due to the deficiency of the enzyme arylsulfatase B that leads to the accumulation of dermatan sulfate in the tissues and its urinary excretion. Mucopolysaccharide deposition leads to a progressive disorder affecting multiple organs that often results in death at a young age. This disease has several oral manifestations, among which dental complications can be serious and include follicles similar to dentigerous cysts, malocclusions, condylar defects and gingival hyperplasia, in addition to a short neck, corneal opacity, macroglossia, skull enlargement, anteroposterior dimension long, claw hand is some of the clinical features. A case of a 14-monthold patient is presented, who attended a pediatric dentistry consultation for episodes of fever, low MANIFESTACIONES ORALES DEL SÍNDROME DE MAROTEAUX-LAMY (MUCOPOLISACARIDOSIS VI) ORAL MANIFESTATIONS OF MAROTEAUX-LAMY SYNDROME (MUCOPOLYSACCHARIDOSIS VI)