Mucopolysaccharidosis type I S; MPS I S

Enzyme replacement therapy for patients with a defect in the enzyme a-L-iduronidase is now possible. This includes individuals with Scheie syndrome, but also Hurler and Hurler-Scheie syndromes.

Early recognition and treatment of spinal cord compression can prevent permanent nerve damage. Treatment is also given for heart problems caused by leaky valves.

The National MPS Society -- www.mpssociety.org

Scheie syndrome is compatible with an almost normal lifespan. However, some disabilities such as limitation of joints, blindness, or deafness are likely to occur later in life.

• Compression of the spinal cord, which can lead to loss of nerve function
• Extremity deformities
• Hearing loss and deafness
• Problems with the aortic valve
• Vision problems

Call your health care provider if you have symptoms of this disorder.

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Scheie syndrome Symptoms

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Scheie syndrome Prevention

Review Date: 05/15/2011
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)