Morquio

Society for Mucopolysaccharide Diseases
MPS House, Repton Place, White Lion Road, Amersham, Buckinghamshire, HP7 9LP, UK
Tel: 0845 389 9901 Fax: 0845 389 9902
National Registered Charity No. 287034

Morquio Disease (MPS IV)

Morquio disease is one of the mucopolysaccharide diseases also known as MPS lV. It takes its name from Dr. Morquio, a paediatrician in Montevideo, Uruguay, who in 1929 described a family of four children affected by the condition.

If you are the parent of a newly diagnosed child, it is important to remember that Morquio disease varies widely in its severity; even children from the same family may be affected differently. A range of possible problems is described, but this does not mean that your child will experience all of them. Some complications arise early in childhood, while others arise much later or may never occur.

What causes Morquio disease?
There is a continuous process in the body of replacing used materials and breaking them down for disposal. Children with Morquio disease are missing an enzyme which is essential in cutting up the mucopolysaccharide called keratan sulphate. The incompletely broken down mucopolysaccharides cannot be used in the proper development of bones and cartilage and remain stored in cells in the body causing progressive damage. Babies may show little sign of the disease but, as more and more cells become damaged, symptoms start to appear.

The usual form of Morquio disease is known as MPS lVA to distinguish it from the much rarer B form which is caused by the lack of a different enzyme. Sufferers from the B form have similar problems, but tend to be less severely affected.

How is Morquio Disease inherited?
We all have genes inherited from our parents which control whether we are tall, short, fair, etc. Some genes we inherit are 'recessive', that is to say we carry the gene but it does not have any effect on our development. Morquio disease is caused by a recessive gene. If an adult carrying the abnormal gene marries another carrier there will be a one in four chance with every pregnancy that the child will inherit the defective gene from each parent and will suffer the disease. There is a two in three chance that unaffected brothers and sisters of Morquio sufferers will be carriers. They can be reassured however that, as the disease is so rare, the chance of having a partner who is another carrier is very slight provided their partner is not a cousin or other close family member. All families of children with Morquio disease should seek further information from their doctor or from a genetic counsellor before planning to have more children. There is a more detailed explanation of this complex subject in a booklet on the pattern of Inheritance available from the MPS Society.

If you already have a child with Morquio disease, it is possible to have tests during a subsequent pregnancy and to be told whether the baby you are carrying is affected. It is important to consult your doctor as soon as you suspect you might be pregnant if you wish tests to be arranged.

Is there a cure?
At present there is treatment for symptoms as they arise but no cure for the underlying disease. Bone marrow transplant was tried but with disappointing results and is not recommended as a treatment for Morquio disease.

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