Scheie syndrome - Treatment

Alternative Names

Mucopolysaccharidosis type I S; MPS I S

Treatment:

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.

Support Groups:

The National MPS Society -- www.mpssociety.org

Expectations (prognosis):

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.

Complications:

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

Calling your health care provider:

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

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.

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