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Hurler syndrome - Treatment

Alternative Names

Alpha-L-iduronate deficiency; Mucopolysaccharidosis type I; MPS I H

Treatment:

Enzyme replacement therapy adds a working form of the missing enzyme to the body.

Bone marrow transplant has been used in several patients with this condition. The treatment has had mixed results.

Other treatments depend on the organs that are affected.

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 outlook. Children with this disease develop nervous system problems, and can die young.

Calling your health care provider:

Call your health care provider if:

You have a family history of Hurler syndrome and are considering having children
Your child begins to show symptoms of Hurler syndrome

Low nasal bridge

Reviewed last on: 5/15/2011
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.

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. 03-5115. February 13, 2007.

Wraith JE. Mucopolysaccharidoses and oligosaccharidoses. In: Fernandes J, Saudubray J-m, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases: Diagnosis and Treatment. 4th ed. New York, NY: Springer;2006:chap 39.

Enzyme

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