Maroteaux–Lamy syndrome, or Mucopolysaccharidosis Type VI (MPS-VI), is an inherited disease caused by a deficiency in the enzyme arylsulfatase B (ARSB).

Because people with MPS-VI lack the ability to break down these GAGs, these chemicals build up in the lysosomes of cells.

[4] Signs are revealed early in the affected child's life, with one of the first symptoms often being a significantly prolonged age of learning how to walk.

By age 10, children often develop a shortened trunk, crouched stance, and restricted joint movement.

[5] Individuals with MPS VI may also experience macrocephaly, hydrocephalus, distinctive coarse facial features, macroglossia, dysostosis multiplex, and hepatosplenomegaly.

Molecular genetic testing can give information about the specific mutation causing MPS-VI, but it is only available at specialized laboratories.

Surgery may be necessary to treat abnormalities such as carpal tunnel syndrome, skeletal malformations, spinal cord compression, hip degeneration, and hernias.

[citation needed] Hydrocephalus may be treated by the insertion of a shunt to drain excess cerebrospinal fluid.

A myringotomy, in which a small incision is made in the eardrum, may be helpful for patients with fluid accumulation in the ears.

[citation needed] Certain medications can be used to treat heart abnormalities, asthma-like episodes, and chronic infections associated with MPS-VI.

Although studies have not revealed an ethnic predisposition, certain groups with a high degree of consanguinity have a higher prevalence of MPS-VI.

[7] It is named after Pierre Maroteaux (1926–2019) and his mentor Maurice Emil Joseph Lamy (1895–1975), both French physicians.

[11] Isabel Bueso, a Guatemalan woman with Maroteaux–Lamy syndrome who has been receiving treatment at UCSF Benioff Children's Hospital, was at risk of deportation from the United States after the Trump administration ended the deferred action program in August 2019.