MPS VI - Maroteaux-Lamy

marot1Maroteaux-Lamy Syndrome is also known as MPS VI. It is one of the rarer mucopolysaccharide disorders. It takes its name from two French Doctors, Dr. Maroteaux and Dr. Lamy, who first described the condition in 1963.

Children with Maroteaux-Lamy are missing an enzyme called N-Acetylgalactosamine 4 sulphatase which is essential in cutting up the mucopolysaccharides called dermatan sulphate.

marot3The Australian prevalence of Maroteaux-Lamy syndrome is 1 in 235,000. A particularly severe form of the disorder has been reported among Australian aborigines. It also occurs in Siamese cats.

Maroteaux-Lamy syndrome is very varied in its severity. There are no tests which can predict whether a child will be mildly or severely affected. It is not possible to give estimates of the likely life span as the disease has been only recently described but it is likely that medical advances and better management will increase life expectancy.

marot2Teenagers will go through the normal stages of puberty although possibly a little later than their peers. Maroteaux-Lamy syndrome does not affect an individual's fertility, but a woman who is severely affected may be advised not to get pregnant because of the risk to her health. All the children will be carriers but none will have the disease unless the other parent is also a carrier.

More information on MPS VI is available from our National Office, including an MPS VI syndrome booklet which can be purchased with the enclosed order form.

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