MPS IV - Morquio

morquioMorquio Syndrome is also known as MPS IV. Morquio Syndrome takes its name from Dr. Morquio, a paediatrician in Montevideo, Uruguay, who in 1929 described a family of four children affected by this condition. As Dr. Brailsford from Birmingham, England also described the same condition in the same year, it is sometimes known as Morquio-Brailsford syndrome.

There are two main forms of Morquio syndrome, MPS IVA and MPS IVB. The usual form of Morquio syndrome is known as MPS IVA to distinguish it from the much rarer B form which is caused by the lack of a different enzyme. Individuals with the B form have similar problems but tend to be more mildly affected.

MPS IVA individuals are missing the enzyme called galactose 6-sulphatase and MPS IVB individuals are missing the enzyme called beta-galactosidase.

However, like the other MPS disorders, MPS IV is very varied in its effects on individuals. The MPS IV gene has now been located and is being studied. It is hoped that increasing knowledge of the mutations within individual families will allow for greater prediction of the severity of the disorder for the affected person as well as providing an accurate basis for carrier detection within the family.

Teenagers will go through the normal stages of puberty although possibly a little later than their peers. Morquio 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.

The Australian prevalence of Morquio A syndrome is 1 in 169,000. There are no current figures available for type B prevalence.

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