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MPS Awareness Day 2008



MPS & Related Diseases > MPS IV

MPS IV

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

There is no magic cure for MPS diseases, but there are ways of managing and treating the problems they cause.

What causes this disease?

mucopolysaccharides are long chains of sugar molecule used in the building of connective tissues in the body.

"saccharide" is a general term for a sugar molecule (think of saccharin)

"poly" means many

"muco" refers to the thick jelly-like consistency of the molecules

There is a continuous process in the body of replacing used materials and breaking them down for disposal. Children with Morquio are missing an enzyme which is essential in cutting up the mucopolysaccharides called keratan sulfate. The incompletely broken down mucopolysaccharides 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.

Click here to visit booklet section and learn more about MPS IV.

All familes of affected children should seek further information from their doctor or a Genetic Counselor.

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