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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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