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Hurler syndrome

Alternative Names: Alpha-L-iduronate deficiency; Mucopolysaccharidosis type I; MPS I; Hurler-Scheie Syndrome (MPS 1 H/S); Scheie Syndrome (MPS1S) Treatment: Enzyme replacement therapy is now possible for patients with a defect in the enzyme a-L-iduronidase. Text Continues Below Bone marrow transplantation can improve some of the symptoms of the disease. To prevent mental retardation, a bone marrow transplant probably should be performed at a very young age. Other treatments depend on the affected organ system. Support Groups: For more information and support, contact one of the following organizations: The National MPS Society: www.mpssociety.org Canadian Society for MPS and Related Diseases: www.mpssociety.ca Society for MPS Diseases: www.mpssociety.co.uk Expectations (prognosis): Hurler syndrome is a disease with a poor prognosis. Children with this disease have progressive neurological impairment. Early death can occur. Calling your health care provider: Call your health care provider if you have a family history of Hurler syndrome and are considering having children, or if your child begins to develop a group of the characteristic signs and symptoms of Hurler syndrome. References: Staba SL, Escolar ML, Poe M, et al. Cord-blood transplants from unrelated donors in patients with Hurler's syndrome. N Engl J Med. 2004 May 6;350(19):1960-9. National Institute of Neurological Disorders and Stroke. Mucolipidoses Fact Sheet. Office of Communications and Public Liaison. Bethesda, MD; Publication No. 05-4899. February 9, 2005.

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