Hunter syndrome

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Hunter syndrome is an inherited disease in which long chains of sugar molecules (mucopolysaccharides) are not broken down correctly and build up in the body.

Mucopolysaccharidosis type II, Iduronate sulfatase deficiency

Hunter syndrome is an inherited condition. Boys are most often affected.

The condition is caused by a lack of the enzyme iduronate sulfatase. Without this enzyme, mucopolysaccharides build up in various body tissues, causing damage.

The early-onset, severe form of the disease begins shortly after age 2. A late-onset, mild form causes less severe symptoms to appear later in life.

Review Date: 05/16/2011
Reviewed By: Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).

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