Mucopolysaccharidosis VI (Maroteaux-Lamy Syndrome)

Abstract

Mucopolysaccharidosis VI was first recognized by Maroteaux and coworkers in 1963 as a Hurler-like syndrome, but with preservation of intelligence and excretion of dermatan sulfate unaccompanied by heparan sulfate. Milder forms of MPS VI have been recognized since the initial description of the disorder. The estimated birth incidence of MPS VI ranges from 1 in 100,000 to 1 in 1,300,000 in various populations.