Hunter syndrome

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Risk factors

By Mayo Clinic staff

The risk of an unborn child acquiring Hunter syndrome is determined by the genetics of the child's mother.

Hunter syndrome is an X-linked recessive disease. This means that women carry the disease and pass it on — most often to their sons — but the mothers aren't affected by the disease themselves. Hunter syndrome is the only type of MPS that can be passed on by a defective gene in the mother alone. In all other types of MPS, the child inherits the syndrome because the same defective gene has been passed on by both parents.

In an X-linked recessive disorder, the mutated gene is located on the X chromosome. In this case, the mother is a carrier, which means she has one mutated gene and one normal gene for the condition.

Girls are less at risk of inheriting this disease because they have two X chromosomes. If one of the X chromosomes is defective, their normal X chromosome can provide a functioning gene. If the X chromosome of a boy is defective, however, there isn't another normal chromosome to compensate for the problem.

A man with an X-linked recessive disorder will pass his normal Y gene to his sons, and none will be affected. He will pass his mutated X gene to his daughters, and they will be carriers. These chances are the same in each pregnancy.

If you have a child with Hunter syndrome or other MPS syndrome, talk to your doctor or a genetic counselor before planning to have more children. If you're a sister or aunt of a person with Hunter syndrome, you may also be a carrier. There are tests for genetic carriers, so you may wish to seek genetic counseling before having children.

DS00790

Aug. 9, 2008

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