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MPS IV is also known as Morquio syndrome and it named after the pediatrician Dr. morquio who described a family of four children affected by this condition in 1929, it called Morquio-Brailsford syndrome after Dr. Brailsford who described the same condition in Birmingham, England in the same year.
The disease occurs when Children 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.
MPS IV is one of the rarest of the MPS diseases in the United States. Reliable incidence figures are not available, but estimates have varied between 1 in 200,000 live births to 1 in 300,000 live births.
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.
MPS IV 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 be affected with the disease.
There is a two in three chance that unaffected brothers and sisters of children with MPS IV will be carriers.
They can be reassured, however, that as the disease is so rare, the chance of marrying another carrier is very slight provided they do not marry a cousin or other close family member.
There is no cure for MPS diseases, but there are ways of managing and treating the problems they cause, In 2014 an (ERT) was approved by the FDA for MPS IVA. Currently there is no treatment for MPS IVB.